Term
| which 2 autoantigens elicit autoantibodies that cause dilated cardiomyopathy? |
|
Definition
| adenine nucleotide translator (ANT), beta-adrenoreceptor |
|
|
Term
| 2 autoantigens which elicit autoantibodies that cause chronic active hepatitis? |
|
Definition
|
|
Term
| 2 autoantigens which elicit autoantibodies that cause primary biliary cirrhosis |
|
Definition
| actin, pyruvate dehydrogenase complex E2 (PDC-E2) |
|
|
Term
| 2 autoantigens which elicit autoantibodies that cause dilated cardiomyopathy |
|
Definition
| adenine nucleotide transporter ANT, beta-adrenoreceptor |
|
|
Term
| 3 autoantigens which elicit autoantibodies that cause autoimmune hepatitis |
|
Definition
| asialoglycoprotein, CYP2D6, actin |
|
|
Term
| autoantigen which elicits autoantibodies that cause crhon's disease |
|
Definition
|
|
Term
| autoantigen which elicits autoantibodies that cause autoimmune coronary artery disease |
|
Definition
|
|
Term
| autoantigen which elicits autoantibodies that cause Guillain Barre syndrome |
|
Definition
|
|
Term
| 3 autoantigens which elicit autoantibodies that cause type 1 DM |
|
Definition
| insulin, IA-2, glutamate decarboxylate GAD65 |
|
|
Term
| 10 autoantigens which elicit autoantibodies that cause SLE |
|
Definition
| collagen (multiple types), carbonic anhydrase 2, cardiolipin, fibronectin, golgin, RNA polymerase, histone H2A-H2B, p53, C1q, insulin receptor |
|
|
Term
| 4 autoantigens which elicit autoantibodies that cause Sjogren's syndrome |
|
Definition
| carbonic anhydrase 2, La phosphoprotein, IgA, golgin |
|
|
Term
| 7 autoantigens which elicit autoantibodies that cause systemic sclerosis |
|
Definition
| carbonic anhydrase 2, collagen, centromere, RNA polymerase, cytokines (il1, il6, il10), fibrillarin, topoisomerase 1 (scl-70) |
|
|
Term
| 6 autoantigens which elicit autoantibodies that cause RA |
|
Definition
| keratin, collagen, fibronectin, glucose-6-phosphate isomerase, golgin, cytokines (il1, il6, il10) |
|
|
Term
| 2 autoantigens which elicit autoantibodies that cause dermatomyositis/polymyositis? |
|
Definition
| aminoacyl-tRNA synthetase, ATPase |
|
|
Term
| 2 autoantigens that elicit autoantibodies that cause vitiligo |
|
Definition
|
|
Term
| autoantigen which elicits autoantibodies that cause Wegener's/Churg-Strauss |
|
Definition
|
|
Term
| which HLA class is associated with SLE and Sjogren's syndrome |
|
Definition
|
|
Term
| which HLA class is associated with RA |
|
Definition
|
|
Term
| which HLA class is associated with Celiac |
|
Definition
|
|
Term
| which HLA class is associated with Psoriasis |
|
Definition
|
|
Term
| which 4 HLA classes are associated with type 1 DM |
|
Definition
|
|
Term
| which 2 HLA classes are associated with Grave's disease |
|
Definition
|
|
Term
| which 2 HLA classes are associated with myasthenia gravis |
|
Definition
|
|
Term
| which HLA class is associated with Systemic sclerosis? |
|
Definition
|
|
Term
| which HLA class is associated with Goodpasturs syndrome? |
|
Definition
|
|
Term
| which HLA class is associated with protection against type 1 DM |
|
Definition
|
|
Term
| what is a sign on CBC of Leukocyte adhesion disease (a primary immunodeficiency)? |
|
Definition
| increased Neutrophils (and wounds w/o pus) |
|
|
Term
| What is a sign on CBC of Wiskott-Aldreich syndrome |
|
Definition
|
|
Term
| What is a sign on CBC of asplenia |
|
Definition
|
|
Term
| What is a sign on CXR for Digeorge syndrome and SCID |
|
Definition
|
|
Term
| What is a sign on CXR for adenosine deamenase deficiency? |
|
Definition
|
|
Term
| What is the prophylactic Tx for patients with severe congenital neutropenia? |
|
Definition
|
|
Term
| what is the Tx for severe congenital neutropenia? |
|
Definition
|
|
Term
| What is the prophylactic Tx for patients with asplenia? |
|
Definition
| Vaccinations and penicillin |
|
|
Term
| What are 2 processes (except the tendency for bacterial and fungal infections) that occur in leukocyte adhesion deficiency? |
|
Definition
| Delayed wound healing and delayed loss of umbilical cord |
|
|
Term
| Which 3 organs are most susceptible to infection in chronic granulomatous disease and why? |
|
Definition
| Organs rich in macrophages: liver, lungs, lymph nodes |
|
|
Term
| 5 typical organisms that cause infection in chronic granulomatous disease? |
|
Definition
| Staph. Aureus, Brukholderia Cepacia, Serratia Meracens, Fungal, Mycobateria |
|
|
Term
| What is the prophylactic Tx of Chronic granulomatous disease |
|
Definition
|
|
Term
| What is the definitive Tx for chronic granulomatous disease? |
|
Definition
|
|
Term
| What is deficient in ‘Mendelian Susceptibility to Mycobacterial Disease’ patients? |
|
Definition
| Il12, il12 receptor, IFN gamma |
|
|
Term
| 3 infections typical of Toll-like receptor deficiencies? |
|
Definition
| Recurrent strep.pneumonia, Staph.Aureus, viral |
|
|
Term
| Deficiency in any complement factor in the lytic pathway (C55-C9) may lead to infection with which organism typically? |
|
Definition
|
|
Term
| Which 2 tests are used to assess the functionality of the alternative and classic pathway? |
|
Definition
|
|
Term
| What is the prophylactic Tx in any complement deficiency? |
|
Definition
| Daily penicillin + vaccinations |
|
|
Term
| What is the deficiency in severe combined immunodeficiency? |
|
Definition
|
|
Term
| 4 most common clinical manifestations of severe combined immunodeficiency? |
|
Definition
| Failure to thrive, oral candidiasis, PCP pneumonia, protracted diarrhea |
|
|
Term
| What is the deficiency in Ataxia telangiectasia? |
|
Definition
| B cells with a progressive T cell loss |
|
|
Term
| How is ataxia telangiectasia diagnosed? |
|
Definition
| cytogenetic analysis showing excessive chromosomal rearrangements (mostly affecting chromosomes 7 and 14) in lymphocytes |
|
|
Term
| What are 3 types of malignancies associated with ataxia telangiectasia? |
|
Definition
| Leukemia, lymphoma and carcinoma |
|
|
Term
| What is the clinical triad of Wiskott Aldrich syndrome? |
|
Definition
| Eczema, thrombocytopenia (bleeding), recurrent bacterial infections |
|
|
Term
| What is the malignancy that may be the consequence of Wiskott Aldrich syndrome? |
|
Definition
| Lymphoma associated with EBV or Kaposi sarcoma associated with HHV8 |
|
|
Term
| How is the diagnosis of wiskott Aldrich done? |
|
Definition
| Intracellular immunofluroscence of WAS protein |
|
|
Term
| What type of cells are predominantly involved in primary immunodeficiencies? |
|
Definition
|
|
Term
| What is a late cardiovascular complication of B lymphocyte deficiency? |
|
Definition
| Cor pulmonale d/t recurrent bronchiectasis |
|
|
Term
| How do you diagnose agammaglobulinemia? |
|
Definition
| intracellular immunofloroscence of monocytes lacking BTK, a kinase involved in the maturation pathway from precursor to mature B cell |
|
|
Term
| What is the definition of agammaglobulinemia? |
|
Definition
| Less than 1% of normal B cells in blood count |
|
|
Term
| How do you make a diagnosis of hyper IgM syndrome? |
|
Definition
| Immunofluorescence of monocytes to show CD40L deficiency of CD40 (receptor) deficiency which interfere with class switching to IgG and IgA |
|
|
Term
| What is a clinical sign of high IgM syndrome? |
|
Definition
|
|
Term
| What is the B lymphocyte count in patients with combined-variable immunodeficiency? |
|
Definition
| Usually normal, may be decreased |
|
|
Term
| Besides infections, what other 5 clinical manifestations may occur in combined-variable immunodeficiency? |
|
Definition
| splenomegaly, granulomatous lesions, colitis, antibody-mediated autoimmune disease, and lymphomas |
|
|
Term
| What is the MC primary immunodeficiency? |
|
Definition
|
|
Term
| Over their lifetime patients with IgA deficiency have an increased susceptibility to what 2 groups of disease? |
|
Definition
| Atopic disorders and autoimmune diseases |
|
|
Term
| In isolated recurrent sinopulmonary infections, what primary immunodeficiency should be sought? |
|
Definition
|
|
Term
| What is the indication to administer immunoglobulin replacement therapy? |
|
Definition
| Recurrent infections with the absence of immunoglobulins |
|
|
Term
| What is a possible fatal adverse effect of immunoglobulin replacement therapy? |
|
Definition
| Anaphylaxis d/t antibody reaction in the totally deficient IgA patient |
|
|
Term
| What is the basic mechanism of Hemophagocytic Lymphohistiocytosis? |
|
Definition
| unremitting activation of CD8+ T lymphocytes and macrophages that leads to organ damage |
|
|
Term
| What is a common trigger of Hemophagocytic lymphohistiocytosis? |
|
Definition
| Viral infection – usually EBV |
|
|
Term
| How is the diagnosis of Hemophagocytic Lymphohistiocytosis made? |
|
Definition
| Clinically: fever, hepatosplenomegay, edema, neurologic diseases, blood cytopenia, increased liver enzymes, hypofibrinogenemia, high triglyceride levels, elevated markers of T cell activation, and hemophagocytic features in the bone marrow or cerebrospinal fluid |
|
|
Term
| What is the Tx of Hemophagocytic lymphohistiocytosis? |
|
Definition
| Immediate immunosuppression with either VP-16 or anti T-cell antibodies. Once remission has been achieved- HSCT |
|
|
Term
| What is autoimmune lymphoproliferative syndrome? |
|
Definition
| Nonmalignant proliferation of B and T cells with splenomegaly and lymphadenopathy |
|
|
Term
| What 4 autoimmune manifestations are common in autoimmune lymphoproliferative syndrome? |
|
Definition
| cytopenias, Guillain-Barré syndrome, uveitis, and hepatitis |
|
|
Term
| What is a hallmark on immunohistochemistry of T cells in autoimmune lymphoproliferative syndrome? |
|
Definition
| CD4-, CD8-, and TCR+ T cells |
|
|
Term
| 4 possible mutations in autoimmune lymphoproliferative syndrome? |
|
Definition
| Fas-ligand, caspase 10, caspase 8, and neuroblastoma RAS viral oncogene homologue (NRAS) |
|
|
Term
| What is the Tx of autoimmune lymphoproliferative syndrome? |
|
Definition
|
|
Term
| What is immunodysregulation polyendocrinopathy enteropathy X-linked syndrome (IPEX)? |
|
Definition
| It is a primary immunodeficiency characterized by causing diffuse enteropathy. The pathophysiology is defect T-reg (CD25) activation or absence |
|
|
Term
| Which loss-of-function mutation underlies immunodysregulation polyendocrinopathy enteropathy X-linked syndrome (IPEX)? |
|
Definition
| FOXP3, transcription factor in the activation of T-reg |
|
|
Term
| What are other diseases associated with syndromic asplenia (primary immunodeficiency)? |
|
Definition
| Heart defects and situs inversus |
|
|
Term
| What are other diseases associated with severe congenital neutropenia, a primary immunodeficiency? |
|
Definition
| Cardiac, urogenital, bone malformations, short stature, exocrine pancreatic insufficiency |
|
|
Term
| What are other disorders associated with leukocyte adhesion disorder, a primary immunodeficiency? |
|
Definition
| Cognitive development disorder and growth retardation |
|
|
Term
| What are other disorders associated with x-linked CGD, a primary immunodeficiency? |
|
Definition
| Specifically those with the McLeod phenotype: anemia, acanthocytosis, a severe risk of immune reaction d/t absence of the kell antigen |
|
|
Term
| What additional 3 disorder are associated with Adenosine Deaminase deficiency (SCID), except for being a primary adaptive immunodeficiency? |
|
Definition
| Chondrodysplasia, cognitive impairment, dysautonomia |
|
|
Term
| A profound T cell immunodeficiency is rarely associated with Di-George syndrome, which 3 additional disorders are associated with this? |
|
Definition
| Conotruncal abnormalities, hypoparathyroidism, dysmorphic syndrome |
|
|
Term
| Primary T cell immunodeficiencies related to impaired calcium channels are also associated with 3 other disorders |
|
Definition
Nonprogressive myopathy manifesting as fatigue
Abnormal teeth
Anhidrotic ectodermal dysplasia |
|
|
Term
| What is the MC primary T/B cell immunodeficiency caused by impaired DNA repair mechanisms? |
|
Definition
|
|
Term
| What is a growth hormone insensitivity syndrome that also encompasses primary adaptive immunodeficiency? |
|
Definition
|
|
Term
| What are the additional syndromic symptoms of hyper-IgE syndrome, a primary adaptive immunodeficiency? |
|
Definition
| facial dysmorphy, defective loss of primary teeth, hyperextensibility, scoliosis, and osteoporosis |
|
|
Term
| 3 additional disorders to Cartilage Hair Hypoplasia disease, a primary adaptive combined B/T cell immunodeficiency syndrome |
|
Definition
| short-limb dwarfism, metaphyseal dysostosis, and sparse hair |
|
|
Term
| A primary adaptive T cell immunodeficiency with panhypoimmunoglobulinemia and hepatic veno-occlusive complications, seen in Lebanese? |
|
Definition
|
|
Term
| 5 drugs that may cause IgA deficiency |
|
Definition
| antimalarials, captopril, penicilla-mine, phenytoin, and sulfazaline |
|
|
Term
| 7 autoimmune diseases associated with Sjogren’s syndrome |
|
Definition
Rheumatoid arthritis
Systemic lupus erythematosus
Scleroderma
Mixed connective tissue disease
Primary biliary cirrhosis
Vasculitis
Chronic active hepatitis |
|
|
Term
| What is the incidence of sjogren’s syndrome |
|
Definition
|
|
Term
| What % of patients with autoimmune rheumatic diseases also suffer secondary sjogrens syndrome |
|
Definition
|
|
Term
| What are 5 predisoposing factors for the development of lymphoma in Sjogren’s syndrome and what blood test correlates with them? |
|
Definition
Persistent parotid gland enlargement, purpura, leukopenia, cryoglobulinemia, and low C4 complement
High levels of il18+ macrophages |
|
|
Term
| Which lymphocytes correlate with mild lesions in sjogrens syndrome, and which with severe lesions? |
|
Definition
T cells – mild
B cells – severe |
|
|
Term
| Patients with sjogren’s syndrome and high levels of the protein b-cell-activating-factor, show which blood dyscrasia? |
|
Definition
|
|
Term
| What is the triggering factor for epithelial activation in Sjogren’s syndrome? |
|
Definition
| Persistent enteroviral infection (Coxsackie B virus) |
|
|
Term
| What may be the underlying cause of the neuroepithelial dysfunction seen in Sjogren’s syndrome? |
|
Definition
| A defect in cholinergic activity and redistribution of aquaporines |
|
|
Term
| SNPs in which 2 genes appear in all patients with Sjogren’s syndrome and account for the abnormal activation of interferon type 1? |
|
Definition
|
|
Term
| Which HLA-class 2 type is widespread in Sjogren’s syndrome patients? |
|
Definition
|
|
Term
| What are the dynamics of Sjogrens syndrome and how long does it usually take to develop full blown disease from the first presentation? |
|
Definition
|
|
Term
| 5 oral symptoms of Sjogren’s syndrome? |
|
Definition
| difficulty in swallowing dry food, inability to speak continuously, a burning sensation, increase in dental caries, and problems in wearing complete dentures |
|
|
Term
| 3 PE findings in Sjogrens syndrome |
|
Definition
Dry, erythematous, sticky oral mucosa
Enlargement of the parotid
Atrophy of the filiform papillae in the dorsum of tongue |
|
|
Term
| 2 steps in the Dx of Sjogren’s syndrome |
|
Definition
1. sialometry, sialography, scintigraphy, US, MRI or MR sialography
2. Bx of the labial minor salivary glands to show lymphocyte infiltration |
|
|
Term
| 8 Ocular symptoms in Sjogrens syndrome |
|
Definition
| sandy or gritty feeling under the eyelids, burning, accumulation of thick strands at the inner canthi, decreased tearing, redness, itching, eye fatigue, and increased photosensitivity |
|
|
Term
| What is the process of keratoconjunctivitis sicca in Sjogrens syndrome |
|
Definition
| Destruction of the corneal and bulbar conjunctivae |
|
|
Term
| 3 diagnostic procedures for keratoconjunctivitis sicca in Sjogren’s syndrome |
|
Definition
1.Tear composition (breakup time, lysozyme)
2.Slit lamp with rose bangal staining – corneal filaments
3.Tear flow – Schirmers test |
|
|
Term
| Which 5 other symptoms/signs may be involved in Sjogren’s syndrome d/t decreased exocrine gland secretions, besides oral and ocular? |
|
Definition
| Dry skin, subclinical pancreatitis, dry nose/trachea/respiratory tract, esophageal mucosal atrophy, dysparenunia – dry external genitelia |
|
|
Term
| Patients with Sjogrens syndrome secondary to RA specifically complain of what 5 symptoms |
|
Definition
| easy fatigability, low-grade fever, Raynaud's phenomenon, myalgias, and arthralgias |
|
|
Term
| what is the kidney involvement in Sjogrens syndrome? |
|
Definition
| interstitial nephritis - hyposthenuria renal tubular dysfunction with or without acidosis |
|
|
Term
| what size vessels are involved in vasculitis associated with Sjogren's syndrome? 4 symptoms typical of this vasculitis? |
|
Definition
small and medium-sized vessels
urticaria, skin ulcerations, purpura, mononeuritis multiplex |
|
|
Term
| sensoryneural hearing loss in Sjogren's syndrome is associated with what serology? |
|
Definition
|
|
Term
| 11 extraglandular (systemic) manifestations in Sjogren's syndrome by decreasing prevalence |
|
Definition
| arthralgia/arthritis, Raynaud's, lymphadenopathy, lung involvement, vasculitis, kidney involvement, liver involvement, lymphoma, splenomegaly, peripheral neuropathy, myositis |
|
|
Term
| what are the neurological symptoms that go along with vasculitis in Sjogren's syndrome |
|
Definition
| hemiparesis, transverse myelopathy, hemisensory deficits, seizures, and movement disorders, aseptic meningitis, multiple sclerosis |
|
|
Term
| 3 bad prognostic factors for lymphoma in Sjogren's syndrome |
|
Definition
| B symptoms, lymph node mass >7 cm in diameter, and high or intermediate histologic grade |
|
|
Term
| what lab finding occurs in 70% of patients with Sjogren's syndrome? |
|
Definition
|
|
Term
| 6 viral infections causing bilateral parotid enlargement |
|
Definition
Mumps
Influenza
Epstein-Barr
Coxsackievirus A
Cytomegalovirus
HIV |
|
|
Term
| 4 metabolic conditions that may cause bilateral parotid enlargement |
|
Definition
Diabetes mellitus
Hyperlipoproteinemias
Chronic pancreatitis
Hepatic cirrhosis |
|
|
Term
| 6 potential items in the diagnosis of Sjogren's syndrome |
|
Definition
need 4 out of 6 with histology or serology being compulsory:
1. ocular symptoms
2. oral symptoms
3. ocular signs: shirmer's test, rose-bangal dye
4. histology - lymphocytes
5. oral signs - scialography, scintography, less than 1.5ml in 15 mins
6. serology: Ro-SSA, La-SSB |
|
|
Term
| 2 ocular Tx in Sjogren's syndrome in case there are corneal ulcerations? |
|
Definition
|
|
Term
| 4 groups of drugs that should be avoided in Sjogren's syndrome b/c they may worsen lacrimal/salivary function? |
|
Definition
| diuretics, antihypertensive drugs, anticholinergics, and antidepressants |
|
|
Term
| what drugs may be used in Sjogren's syndrome to stimulate secretions? |
|
Definition
|
|
Term
| what is the Tx of arthralgia in sjogren's syndrome |
|
Definition
|
|
Term
| what is the only use of glucocorticosteroids and/or immunosuppressive drugs in Sjogren's syndrome |
|
Definition
|
|
Term
| what is the Tx of renal tubular acidosis in patients with Sjogren's syndrome |
|
Definition
|
|
Term
| what is the Tx of Sjogren's syndrome induced lymphoma? |
|
Definition
|
|
Term
| what is an effective Tx of arthralgia, vasculitis and fatigue in Sjogren's syndrome? |
|
Definition
|
|
Term
| 3 hormones that may cause anaphylaxis |
|
Definition
| insulin, vasopressin, parathormone |
|
|
Term
| 4 enzymes that may cause anaphylaxis |
|
Definition
| trypsin, chemotrypsin, penicillinase, streptokinase |
|
|
Term
| what is the component in vaccine preservatives that causes anaphylaxis |
|
Definition
| polysacharrides: dextran, thiomersal |
|
|
Term
| 5 Abx that may cause anaphylaxis |
|
Definition
| nitrofurantoin, cephalosporins, quinolones, amphotericin B, penicilline |
|
|
Term
| 3 chemotherapeutic agents that may cause anaphylaxis |
|
Definition
| carboplatin, paclitaxel, doxorubicin |
|
|
Term
| which groups of drugs may cause anaphylaxis? |
|
Definition
| antibiotics, chemotherapeutic agents, protamine, muscle relaxants (recoronium), diagnostic agents, local anaesthetics (lidocaine) |
|
|
Term
| 2 vitamins that may cause an anaphylactic reaction |
|
Definition
|
|
Term
| how long does urticaria as part of anaphylaxis last? |
|
Definition
|
|
Term
| what is a gross and microscopic finding in the alveoli during anaphylaxis? |
|
Definition
|
|
Term
| 3 microscopic findings in the bronchi during anaphylaxis? |
|
Definition
vascular congestion
eosinophilic infiltration
separation of the collagen fibers from the glandular elements |
|
|
Term
| how would you distinguish anaphylaxis from bronchoconstriction caused by the ingestion of NSAIDs or Aspirin? |
|
Definition
| tryptase nor IgE are elevated. it is not IgE mediated |
|
|
Term
| what is a finding on PE typical of the bronchoconstriction syndrome caused by ingestion of NSAIDs or Aspirin? |
|
Definition
|
|
Term
| when can elevated levels of tryptase be noticed in the blood following an anaphylactic reaction? |
|
Definition
| within 4 hrs of the reaction |
|
|
Term
| what are the 4 steps in the treatment of anaphylaxis? |
|
Definition
1. 2.5 ml IV Epinephrine every 10 mins
if hypotensive:
2. dopamine
3. fluid rescucitation
if hypoxic:
4. add antihistamines: 50mg IV/IM Diphenhydramine or 250mg IV aminophylline |
|
|
Term
| what are 2 possible complications of anaphylaxis indicated by unresolved hypoxemia under epinephrine? |
|
Definition
| cardiac arrhythmia and/or bronchospasm |
|
|
Term
| which medication is contraindicated in the person predisposed to an anaphylactic reaction? in which 2 specific circumstances does this apply? |
|
Definition
beta blockers
allergic to hymenoptera venom or on immunotherapy for allergy manifesting as respiratory problems |
|
|
Term
| what are the hymenoptera venoms which may cause anaphylactic reaction |
|
Definition
| yellow jacket, yellow and baldfaced hornets, paper wasp, honey bee, imported fire ants |
|
|
Term
| what is the management of a patient about to be starting a drug known to potentially cause anaphylaxis? |
|
Definition
|
|
Term
| what % of the (american) population is allergic and may experience anaphylaxis to penicillin? |
|
Definition
|
|
Term
| what 2 materials may be used to perform a skin test in a patient about to start penicillin? |
|
Definition
| benzylpenicilloyl-polylysine (BPL) and the minor determinant mixture (MDM) of benzylpenicillin |
|
|
Term
| what is the management of a patient who must receive a drug known to cross react with a drug that had previously elicited an anaphylactic reaction? |
|
Definition
| desensetization: a dose below the threshold of anaphylaxis, doubling it until reaching therapeutic levels |
|
|
Term
| what is the difference btwn urticaria and angioedema? |
|
Definition
| urticaria involves the superficial dermis whereas angioedema involves as deep as the subcutaneous tissues |
|
|
Term
| what is the timeline for chronic urticaria/angioedema? |
|
Definition
|
|
Term
| what is the MC etiology of chronic urticaria? |
|
Definition
|
|
Term
| 5 possible mechanism of urticaria |
|
Definition
| IgE mediated, bradykinin mediated, complement mediated, direct mast cell degranulation agents, arachidonic acid metabolism affecting agents |
|
|
Term
| what mechanism is responsible for physically induced urticaria? and what 3 physical irritants are those? |
|
Definition
IgE mediated
solar, cold, dermographism |
|
|
Term
| what is the mechanism that is responsible for autoimmune induced urticaria |
|
Definition
|
|
Term
| 2 conditions causing bradykinin-mediated urticaria |
|
Definition
1. hereditary/acquired C1 inhibitor deficiency
2. ace inhibitors |
|
|
Term
| 3 conditions causing complement-mediated urticaria |
|
Definition
serum sickness
transfusion related
necrotizing vasculitis |
|
|
Term
| 4 direct mast cell degranulation drug groups |
|
Definition
| Abx, opiates, contrast, curare |
|
|
Term
| which mechanism of urticaria differs in the timeline of the lesions than the rest of the mechanisms |
|
Definition
| physical - go away in 2 hrs |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| which autoimmune disease is associated with chronic urticaria? |
|
Definition
|
|
Term
| what is the Tx of inherited C1 inhibitor deficiency? |
|
Definition
|
|
Term
| what 3 drugs may be added to the Tx of urticaria in case it becomes persistent |
|
Definition
| hydroxychloroquine, dapsone, or colchicine |
|
|
Term
| 5 organs in which mast cell expansion can be found in systemic mastocytosis |
|
Definition
| BM, spleen, liver, skin, GI mucosa |
|
|
Term
| what type of changes occur in the liver, spleen and BM but not in the skin and the GI system in systemic mastocytosis |
|
Definition
|
|
Term
| what is Darier's sign in urticaria pigmentosa (cutaneous mastocytosis)? |
|
Definition
| mast cell papules/macules turn into urticaria if traumatized |
|
|
Term
| what are the components of the diagnosis of systemic mastocytosis |
|
Definition
| one major criterion + one minor criterion or 3 minor criteria |
|
|
Term
| what is the major criterion in the diagnosis of mastocytosis |
|
Definition
| Bx of other bone marrow or other extracutaneous tissues showing mast cells with confiramation of immunodetection of tryptase or metachromasia |
|
|
Term
| 4 minor criteria of systemic mastocytosis |
|
Definition
1. abnormal mast cell morphology - spindle shape and/or multilobed or eccentric nucleus
2. expression of CD25 (il2 receptor), CD2, CD117 (c-kit)
3. detection of mutation
4. serum tryptase > 20 ng/ml |
|
|
Term
| what % of patients with allergic rhinitis express asthma and vice versa? |
|
Definition
|
|
Term
| 4 allergens that cause perennial allergy |
|
Definition
| animal dander, cockroach-derived proteins, mold spores, or dust |
|
|
Term
| in a person suffering an acute episode of rhinitis with no prior history of allergy, what 2 conditions should be ruled out? |
|
Definition
| foreign body and anatomic defect |
|
|
Term
| what 6 conditions should be excluded in the management of chronic allergic rhinitis? |
|
Definition
| nasal polyps, anatomic defects, sinusitis, GERD, foreign body, medications |
|
|
Term
| in the workup of chronic allergic rhinitis, after excluding other conditions what are the next 2 steps? |
|
Definition
1. evaluation of allergy
2. assess for asthma |
|
|
Term
| what is the Tx of mild intermittent chronic allergic rhinitis |
|
Definition
| intranasal/oral antihistamines, decongestants, cromolyn |
|
|
Term
| what is the Tx of mild persistent or mederately/severe intermittent chronic allergic rhinitis? |
|
Definition
| intranasal corticosteroids |
|
|
Term
| what is the Tx of moderate persistent chronic allergic rhinitis? |
|
Definition
|
|
Term
| what is the Tx of severe persistent chronic allergic rhinitis? |
|
Definition
| oral corticosteroids (3-7 days) |
|
|
Term
| which gene mutation/protein deficiencies confer the highest risk for SLE? |
|
Definition
|
|
Term
| which SNP is associated with a severe manifestation of SLE? |
|
Definition
|
|
Term
| 3 SNPs which correlate with lupus nephritis? |
|
Definition
| anti-DNA, anti-phosopholipid, FCGRIIA |
|
|
Term
| what type of medication may increase the risk of SLE? |
|
Definition
|
|
Term
| what type of chromosomal abnormality may increase the risk of SLE? |
|
Definition
| kleinfelter's XXY (TRAX1 is located on the X chromosome) |
|
|
Term
| 4 environmental factors that increase the risk of SLE? |
|
Definition
| smoking, silica dust, EBV, UV light |
|
|
Term
| what can be seen on Bx of the affected skin in an SLE patient? |
|
Definition
| immune complex deposition and T cell inflammation in the Dermo-Epidermal Junction |
|
|
Term
| which repeated negative blood test are suggestive of another diagnosis than SLE? |
|
Definition
|
|
Term
| what are the 11 diagnostic criteria for SLE? how many out of those would you need to determine a definite diagnosis vs. a possible one? |
|
Definition
4
malar rash, discoid rash, photosensitivity, oral ulcers, hematologic disorder, immunologic disorder, antinuclear antibodies, renal dysfunction, serositis, neurologic dysfunction, nonerosive arthritis |
|
|
Term
| what is considered renal dysfunction in the diagnosis of SLE? |
|
Definition
| proteinuria 0.5g/d or renal casts |
|
|
Term
| what is considered an hematologic disorder in the diagnosis of SLE? |
|
Definition
| hemolytic anemia, leukopenia<4000, lymphopenia<1500 or thrombocytopenia<100,000 w/o any offending drugs |
|
|
Term
| what is considered a neurological dysfunction in the diagnosis of SLE? |
|
Definition
|
|
Term
| what are 2 manifestations of serositis considered in the diagnosis of SLE? |
|
Definition
| pericarditis and serositis |
|
|
Term
| what is the discoid rash in SLE? |
|
Definition
| circular erythematotic patch with scaling |
|
|
Term
| what is considered photosensitivity in SLE? |
|
Definition
| exposure to sun causes rash |
|
|
Term
| what is considered arthritis in SLE? |
|
Definition
| at least 2 joints involved |
|
|
Term
| what is considered an immunologic disorder in the diagnosis of SLE? |
|
Definition
| positive Anti-dsDNA, anti-Sm, and/or anti-phospholipid |
|
|
Term
| what blood tests would you do in the initial evaluation of a patient presenting with symptoms of SLE? |
|
Definition
|
|
Term
| what is the next step in the workup of a patient suspected clinically of SLE with negative initial blood tests? |
|
Definition
| repeat ANA, add anti-dsDNA, anti-Ro |
|
|
Term
| what is the management of a patient positive for ANA or other indicative blood findings and suggestive symptoms that doesn't fulfill 4 criteria of definite SLE? |
|
Definition
| still goes to treatment for SLE |
|
|
Term
| what is the MC symptom of SLE? |
|
Definition
|
|
Term
| what is the MC cutaneous symptom of SLE? |
|
Definition
|
|
Term
| what is the MC hematologic disorder in SLE? |
|
Definition
|
|
Term
| what is the MC neurologic disorder in SLE? |
|
Definition
|
|
Term
| what is the MC cardiopulmonary manifestation of SLE? |
|
Definition
|
|
Term
| 3 most common areas of arthritis in SLE? |
|
Definition
|
|
Term
| how would you distinguish SLE from RA according to joint x-ray? |
|
Definition
| in SLE the arthritis is non-erosive |
|
|
Term
| what % of patients with a discoid lupus-like rash actually have SLE? |
|
Definition
|
|
Term
| what is the leading cause of death in the first decade of SLE? |
|
Definition
|
|
Term
| what happens to patients with SLE who are diagnosed with diffuse proliferative GN (Class 4) and are not treated for it? |
|
Definition
| they develop ESRD in 2 yrs |
|
|
Term
| in diffuse proliferative GN in SLE, when is glucocorticoid and cytotoxic therapy contraindicated? |
|
Definition
| irreversible damage to over 90% of the glomeruli |
|
|
Term
| what is the worst type of lupus nephritis? |
|
Definition
| class 4 - diffuse proliferative GN |
|
|
Term
| what is a concern after several years of suffering class 4 diffuse proliferative GN in SLE? |
|
Definition
| atherosclerotic disease - patients should be balanced for hypertension, hyperlipidemia, hyperglycemia, systemic inflammation |
|
|
Term
| what is the most important question to figure out in a patient suffering neurological symptoms with SLE? |
|
Definition
| are the symptoms caused by a diffuse process requiring immunosupression, are they caused by veno-occlusive disease requiring anticoagulation or are they adverse effects from the treatment |
|
|
Term
| 7 risk factors for atherosclerosis in a patient with SLE |
|
Definition
| older age, hypertension, dyslipidemia, dysfunctional proinflammatory high-density lipoproteins, repeated high scores for disease activity, high cumulative or daily doses of glucocorticoids, and high levels of homocysteine |
|
|
Term
| statin is effective in reducing cardiac events in SLE patients of what only subgroup? |
|
Definition
| patients who underwent renal transplantation |
|
|
Term
| what are 3 life-threatening pulmonary manifestations in SLE? |
|
Definition
| interstitial inflammation leading to fibrosis, shrinking lung syndrome, and intra-alveolar hemorrhage |
|
|
Term
| what is the Tx of mild pleuritis in SLE? |
|
Definition
|
|
Term
| what is the MC cardiac manifestation in SLE? what are 2 serious cardiac manifestations in SLE? |
|
Definition
pericarditis
libman sacks endocarditis, myocarditis |
|
|
Term
| what is the therapy of leukopenia/lymphopenia in SLE? |
|
Definition
| not required, doesn't lead to infection |
|
|
Term
| when should thrombocytopenia be treated in a patient with SLE? |
|
Definition
|
|
Term
| what are 2 serious ocular manifestations in SLE that may cause blindness |
|
Definition
| optic neuritis and retinal vasculitis |
|
|
Term
| what are 2 ocular complications of SLE treatment? |
|
Definition
|
|
Term
| can ANA negative lupus exist? |
|
Definition
| yes but it is rare. ANA would usually be detected until onset of symptoms and if not than at least a year after |
|
|
Term
| what are 2 diagnostic criteria for anti-phospholipid syndrome associated or not with SLE? |
|
Definition
| at least one event of clotting or fetal loss plus 2 positive aPL tests two weeks apart |
|
|
Term
| what 2 blood markers may predict a flare of nephritis or vasculitis in SLE patients? |
|
Definition
elevated dsDNA
decreased C3/C4 |
|
|
Term
| what is a feared adverse effects of antimalarials in SLE patients? |
|
Definition
|
|
Term
| withdrawal from which drug used to treat SLE increases the frequency of flare-ups in SLE? |
|
Definition
|
|
Term
| what is the preventive measure of patients taking anti-malarials for SLE? |
|
Definition
| annual retinal examinations |
|
|
Term
| what is the treatment of severe lupus nephritis? |
|
Definition
| high dose prednisone + cyclophosphamide or mofetil mycofenolate for 5 years |
|
|
Term
| What % of patients treated for lupus nephritis improve? What % of those experience flare ups after 5 yrs of treatment? |
|
Definition
|
|
Term
| What is the induction therapy of choice for proliferative GN in SLE with crescents? What are 2 other options? |
|
Definition
| Mycophenolate mofetil and prednisone. Cyclophosphamide, cyclosporine |
|
|
Term
| What is the effect of SLE on fertility? |
|
Definition
|
|
Term
| Which 3 factors are associated with higher fetal mortality in SLE? |
|
Definition
| Lupus nephritis, anti-phospholipid antibodies, active disease |
|
|
Term
| Which drugs for the treatment of SLE are category A in pregnancy? |
|
Definition
|
|
Term
| Which 3 drugs for the treatment of SLE are category C in pregnancy? And what does it mean? |
|
Definition
|
|
Term
| Which 4 drugs for the treatment of SLE are category D in pregnancy? And what does it mean |
|
Definition
azathioprine, hydroxychloroquine, mycophenolate mofetil, and cyclophosphamide
proven teratogenic in humans, but still may be used in certain situations |
|
|
Term
| Which drug for the treatment of SLE is category X? risk outweighs benefits |
|
Definition
|
|
Term
| 3 fetal outcomes of prednisone during pregnancy |
|
Definition
| low birth weight, developmental abnormalities in the CNS, and predilection toward adult metabolic syndrome |
|
|
Term
| What is the treatment of choice for SLE in pregnancy? |
|
Definition
|
|
Term
| Which treatment in pregnant SLE patients increases the rates of live births? |
|
Definition
| Low dose aspirin + Clexane |
|
|
Term
| why does the presence of anti-Ro antibodies in the mother present a problem to the fetus? |
|
Definition
| Neonatal lupus: rash and congenital heart block |
|
|
Term
| what is the mgmt of a patient with SLE and APS? |
|
Definition
lifetime anticoagulation
1st event: INR 2-2.5
recurrent events: INR 3-3.5 |
|
|
Term
| 3 options for the Tx of treatment-resistant lupus dermatitis? |
|
Definition
| topical tacrolimus or systemic dapsone or thalidomide |
|
|
Term
| what % of patients with SLE experience remissions? |
|
Definition
|
|
Term
| which drugs may cause drug-induced lupus? |
|
Definition
antiarrhythmics procainamide, disopyramide, and propafenone;
the antihypertensive hydralazine; several angiotensin-converting enzyme inhibitors and beta blockers; the antithyroid propylthiouracil; the antipsychotics chlorpromazine and lithium; the anticonvulsants carbamazepine and phenytoin; the antibiotics isoniazid, minocycline, and macrodantin; the antirheumatic sulfasalazine; the diuretic hydrochlorothiazide; the antihyperlipidemics lovastatin and simvastatin; and interferons and TNF inhibitors |
|
|
Term
| how is the Dx of antiphospholipid made? |
|
Definition
| one clinical and one laboratory criterion |
|
|
Term
| what are the clinical criteria in the diagnosis of antiphospholipid syndrome? |
|
Definition
(1) vascular thrombosis defined as one or more clinical episodes of arterial, venous, or small vessel thrombosis in any tissue or organ;
(2) pregnancy morbidity, defined as (a) one or more unexplained deaths of a morphologically normal fetus at or beyond the tenth week of gestation, or (b) one or more premature births of a morphologically normal neonate before the thirty-fourth week of gestation because of eclampsia, severe preeclampsia, or placental insufficiency; or (c) three or more unexplained consecutive spontaneous abortions before the 10th week of gestation |
|
|
Term
| what are the 3 laboratory criteria in the diagnosis of antiphospholipid syndrome? |
|
Definition
anticardiolipin
lupus anticoagulant
anti-b2GPI antibodies |
|
|
Term
| what is the typical complaint of patients with RA? |
|
Definition
| early morning joint stiffness lasting more than 1 hour and easing with physical activity |
|
|
Term
| Which joints are primarily involved in RA |
|
Definition
| Small joints of the hands/feet – symmetric |
|
|
Term
| Which 3 joints are MC involved in RA |
|
Definition
| the wrists, metacarpophalangeal (MCP), and proximal interphalangeal (PIP) |
|
|
Term
| What constitutes swan neck deformity? |
|
Definition
| Hyperextension of the PIP with flexion of the DIP |
|
|
Term
| What constitutes boutennier’s deformity? |
|
Definition
| Flexion of PIP with hyperextension of DIP |
|
|
Term
| Which joints are affected later in the course of RA? |
|
Definition
| Large joints, ankle, midtarsal regions causing flat foot |
|
|
Term
| Which joint in the spine is may be involved in RA? |
|
Definition
| Atlantoaxial leading to neurological deficits |
|
|
Term
| What are the 4 MC observed extra-articular manifestations in RA? |
|
Definition
| Subcutaneous nodules, secondary Sjögren's syndrome, pulmonary nodules, and anemia |
|
|
Term
| 3 characteristics typical of patients with RA who are more likely to develop extra-articular manifestations? |
|
Definition
| Positive RF, early onset physical disability, smoking |
|
|
Term
| Where do the subcutaneous nodules of RA originate? |
|
Definition
| Periosteum, tendons or bursae. In skeletal elements that are susceptible to trauma such as the forearm, Achilles tendon and sacrum |
|
|
Term
| Besides skeletal elements which other 3 tissues may harbor nodules in RA? |
|
Definition
| Pleura, pericardium, peritoneum |
|
|
Term
| 2 characteristics of subcutaneous nodules in RA? |
|
Definition
|
|
Term
| What % of patients with RA have secondary Sjogren’s syndrome? |
|
Definition
|
|
Term
| 6 possible cardiac manifestations in RA? |
|
Definition
| Pericarditins, cardiomyopathy, myocarditis, arrhythmia, mitral regurgitation, ischemic heart disease |
|
|
Term
| 3 characteristics that are seen in RA patients with rheumatoid vasculitis? |
|
Definition
| Long standing disease, positive RF, hypocomplementemia |
|
|
Term
| What is the most severe form of rheumatoid vasculitis? |
|
Definition
| Painful ulcerations appearing on the lower extremities |
|
|
Term
| What is the clinical triad of Felty’s syndrome apparent in less than 1% of patients with RA? |
|
Definition
| neutropenia, splenomegaly, and nodular RA |
|
|
Term
| What differs Felty’s syndrome and T-LGL leukemia in patients with RA? |
|
Definition
| Felty’s syndrome presents in advance-staged patients, whereas, T-LGL leukemia may present early in the disease |
|
|
Term
| RA patients have a higher risk for which malignancy? Which subset of patients with RA may develop it? |
|
Definition
Lymphoma
Felty’s syndrome, highly active disease |
|
|
Term
| What is the MCC of death in patients with RA? |
|
Definition
|
|
Term
| Which endocrine abnormality may be part of the pathogenesis of RA? |
|
Definition
| Hypogonadism, specifically low levels of testosterone |
|
|
Term
| Which 3 genetic components contribute to the risk of RA |
|
Definition
1. MHC class 2 – HLA DRB1 – 3rd hypervariable region called shared epitope encoding for anti-CCP antibodies
2. PTPN22 – encoding for lymphoid tyrosine phosphatase, a protein that regulates T and B cell function.
3. Different SNPs detected by genome-wide association studies (GWAS) |
|
|
Term
| What is the MC environmental risk of RA? |
|
Definition
|
|
Term
| RA associated with smoking usually shows which markers? |
|
Definition
|
|
Term
| Which 3 viruses have been implicated in the pathogenesis of RA, but could not be proved as causative agents? |
|
Definition
| EBV, mycoplasma and parvovirus B19 |
|
|
Term
| What component of the joint is primarily involved in RA? |
|
Definition
|
|
Term
|
Definition
| Hyperplasia of the synovial cells that invade to adjacent bone and cartilage elements in the joint |
|
|
Term
| Which protein provides the invasive ability of the synovial cell which predominates in the pannus? |
|
Definition
|
|
Term
| What 6 cell types comprise the infiltrate in RA? |
|
Definition
| T cells, B cells, plasma cells, dendritic cells, mast cells, and a few granulocytes |
|
|
Term
| Which is the predominate cell type in the infiltrate of RA? |
|
Definition
|
|
Term
| Which other conditions might RF be positive besides in RA? |
|
Definition
| Sjögren's syndrome, SLE, cryoglobulinemia, subacute bacterial endocarditis and hepatitis B and C |
|
|
Term
| 2 serum antibodies to be checked in suspected RA? |
|
Definition
|
|
Term
| Which is the most specific antibody for RA? |
|
Definition
|
|
Term
| What is the predominant cell in the synovial fluid vs. the synovial tissue in RA? |
|
Definition
|
|
Term
| How can synovial fluid aspiration differentiate btwn inflammatory and osteoarthritic joint condition? |
|
Definition
| In RA (inflammatory) the WBC count will typically be 5000-50,000. Whereas, in osteoarthritis the count will be under 2000 |
|
|
Term
| What are 3 findings on plain radiography in RA? |
|
Definition
| soft tissue swelling, symmetric joint space loss, and subchondral erosions |
|
|
Term
| What finding on MRI may precede the appearance of joint erosion on both MRI and plain radiography? |
|
Definition
|
|
Term
| What % of patients will go into diagnosed with RA will undergo spontaneous remission |
|
Definition
|
|
Term
| What is the MC dynamic of RA? |
|
Definition
| A progressive disease with wax and wane dynamics (no complete remissions) |
|
|
Term
| What is the effect of RA on the median life expectancy of males and females? |
|
Definition
| Shortens by 7 yrs in males and 3 yrs in females |
|
|
Term
| 5 characteristics of patients with higher risk for increased mortality with RA? |
|
Definition
| systemic extraarticular involvement, low functional capacity, low socioeconomic status, low education, and chronic prednisone use |
|
|
Term
| 2 serious adverse effects of hydroxychloroquine |
|
Definition
| Irreversible retinal damage, cardiotoxicity |
|
|
Term
| 2 serious adverse effects of sulfasalazine |
|
Definition
| Granulocytopenia and hemolytic anemia (with G6PD def) |
|
|
Term
| 2 DMARDs for which you should check LFTs prior to initiating therapy? |
|
Definition
| MTX, Leflunomide, Sulfasalazine |
|
|
Term
| 3 DMARDs for which you should check viral hepatitis serologies prior to initiating therapy |
|
Definition
| MTX, Leflunomide, Rituximab |
|
|
Term
| 5 DMARDs for which you should perform CBC prior to therapy initiation? |
|
Definition
| MTX, Leflunomide, Sulfasalazine, Rituximab, Anakinra (IL-1 receptor antagonist) |
|
|
Term
| Which DMARD causes increased LDL cholesterol? |
|
Definition
| Tocilizumab (anti il-6 receptor) |
|
|
Term
| Which 3 types of DMARDs cause increased infections and may require preliminary PPD testing? |
|
Definition
| Anti TNFs, Anakinra (il1 receptor antagonist), Tocilizumab (anti il6 receptor) |
|
|
Term
| What is a serious fatal condition that may occur d/t treatment with Rituximab? |
|
Definition
|
|
Term
| Which types of patients with RA benefit more from Rituximab? |
|
Definition
| Patients with seropositive disease |
|
|
Term
| What is the role of NSAIDs in RA? |
|
Definition
| Adjunctive (not mainstay) – mgmt. of symptoms uncontrolled by other measures |
|
|
Term
What is the role of NSAIDs in RA? Adjunctive (not mainstay) – mgmt. of symptoms uncontrolled by other measures
3 uses of glucocorticoids in the Tx of RA? |
|
Definition
1. Bridging therapy (low/moderate dose)
2. Acute episodes
3. Chronic disease with failure to control via DMARDs |
|
|
Term
| What and when is a preventive treatment given in the setting of glucocorticoid treatment in autoimmune diseases |
|
Definition
| When treatment is given at a dose of 5 mg/d for over 3 months, bisphosphonates should be administered to prevent osteoporosis |
|
|
Term
| Which DMARD has not been shown to delay radiographic progression of disease and thus is not considered to be a true DMARD? |
|
Definition
|
|
Term
| What is the role of hydroxychloroquine in the Tx of RA? |
|
Definition
| Mild early disease or adjunctive |
|
|
Term
| 4 criteria in the Dx of RA? |
|
Definition
number/type of joint involvement
serology
duration over/under 6 wks
acute phase reactants |
|
|
Term
| which DMARD may cause stomatittis/mouth ulcers? |
|
Definition
|
|
Term
| which 2 DMARDs may cause alopecia? |
|
Definition
|
|
Term
| 2 CIs to use of anti-TNF agents in RA? |
|
Definition
active infection
hypersensitivity reaction to any of these agents |
|
|
Term
| 5 anti-TNF agents used in the Tx of RA? |
|
Definition
| adalimumab, golimumab, (TNF rides a limo chased by a mob) infliximab, etanercept, certolizumab |
|
|
Term
| what is the cutoff in PPD skin test for initiating workup for active TB in patients destined to undergo anti-TNF Tx? |
|
Definition
|
|
Term
| which DMARD should not be given in combination with anti-TNF agents in RA and why? |
|
Definition
| Anakinra, d/t increased risk of infection |
|
|
Term
| how are anti-TNF agents usually administered in RA? |
|
Definition
|
|
Term
| what is the general treatment approach to the patient with RA? |
|
Definition
| treat early and aggressively to avoid joint damage |
|
|
Term
| what is the initial treatment of choice for moderate to severe RA? |
|
Definition
|
|
Term
| what is the next step in Tx of MTX failure RA? 3 options |
|
Definition
combination therapy:
methotrexate, sulfasalazine, and hydroxychloroquine (triple therapy); methotrexate and leflunomide; and methotrexate plus a biological |
|
|
Term
| 3 factors associated with developing structural joint injuries in RA |
|
Definition
| elevated serum level of acute-phase reactants, high burden of joint inflammation, and the presence of erosive disease |
|
|
Term
| what are the rates of complete remission in RA? |
|
Definition
|
|
Term
| 3 parameters to determine remission in RA? |
|
Definition
one or less joints involved - tender+swollen
CRP<1
patient global assessment =<1 (0-10) |
|
|
Term
| 4 indications for MCP arthroplasty (hand) with silicon in RA |
|
Definition
| severe decreased arc of motion, marked flexion contractures, MCP joint pain with radiographic abnormalities and severe ulnar drift |
|
|
Term
| what are 2 surgical options for rheumathoid wrist in RA after failure of medical Tx? |
|
Definition
| Synovectomy and limited fusion |
|
|
Term
| 3 indications for Arthrodesis/arthroplasty and total wrist arthroplasty in RA? |
|
Definition
| substantial wrist pain and functional impairment in severe disease and hallux valgus |
|
|
Term
| what happens to females with RA during pregnancy? |
|
Definition
| 75% undergo overall improvement but most will develop a flair-up post delivery |
|
|
Term
| which medications for RA are allowed during pregnancy? |
|
Definition
| prednisone, hydroxychloroquine, sulfasalazine |
|
|
Term
| what is the role of inheritance in acute rheumatic fever? |
|
Definition
| HLA class 2 and a protein present on B cells - D8-17 increase the risk. present in 3-6% of the population |
|
|
Term
| the cross-reactivity in acute rheumatic fever is btwn which 2 components in the group A strep and which 7 components in the human body? |
|
Definition
M protein and the N-acetylglucosamine
myosin, tropomyosin, keratin, actin, laminin, vimentin, and N-acetylglucosamine |
|
|
Term
| what are 2 exceptions to the timeline it takes acute rheumatic fever to manifest itself? |
|
Definition
| usually 3 wks after infection except indolent carditis and chorea which may manifest 6 months after infection |
|
|
Term
| what is the MC clinical presentation of acute rheumatic fever? |
|
Definition
|
|
Term
| what % of patients with acute rheumatic fever progress to rheumatic heart disease |
|
Definition
|
|
Term
| which valve is almost always involved in rheumatic heart disease? |
|
Definition
|
|
Term
| what is the typical valvular abnormality early in the onset of rheumatic heart disease? |
|
Definition
| mitral regurgitation (stenosis may appear later in disease) |
|
|
Term
| to qualify as a major manifestation, joint involvement in acute rheumatic fever must include these 4 elements |
|
Definition
arthritic vs. only arthralgic
polyarthritic vs. monoarthritic |
|
|
Term
| to be considered a minor manifestation, joint involvement in acute rheumatic fever, joint involvement should include |
|
Definition
| Arthralgia without objective joint inflammation usually affects large joints in the same migratory pattern as polyarthritis |
|
|
Term
| what are 2 characteristics of joint involvement whether considered to be major or minor in acute rheumatic fever |
|
Definition
involves large joints: knee, hip, ankle, elbow
asymmetric |
|
|
Term
| the joint manifestations of acute rheumatic fever are highly responsive to salicylates/NSAIDs. how does this affect the mgmt of joint involvement in the settings of suspected ARF? |
|
Definition
| before Dx avoid use of NSAIDs/Salicylates. if arthritis unresponsive to NSAIDs/Salicylates after 2 days, suspect other Dx |
|
|
Term
| how long is the course of chorea in acute rheumatic fever? |
|
Definition
| 6 wks. resolves by itself |
|
|
Term
| what 2 parts of the body are affected by sydenham's chorea in acute rheumatic fever |
|
Definition
| head (tongue) and upper extremities |
|
|
Term
| 3 elements in the setting of sydenham's chorea as a manifestation of acute rheumatic fever? |
|
Definition
| latent to the infection, no previous manifestations of ARF, found mainly in females |
|
|
Term
| what are the 2 skin manifestations of acute rheumatic fever? |
|
Definition
| erythema marginatum and subcutaneous nodules |
|
|
Term
| 5 characteristics of erythema marginatum in acute rheumatic fever? |
|
Definition
| pink macules, clear center, serpiginous, spreading edge, appearing and disappearing before examiner's eyes, involves trunk and extremities (never face) |
|
|
Term
| 4 characteristics of subcutaneous nodules in acute rheumatic fever |
|
Definition
0.5-2cm
overlying bony areas
go away within 3 wks
painless |
|
|
Term
| subcutaneous nodules in acute rheumatic fever are usually associated with which other manifestation? |
|
Definition
|
|
Term
| what blood tests are used in the diagnosis of acute rheumatic fever to prove previous strep A infection |
|
Definition
| serologies: ASO, anti-DNAase-B (ADB) |
|
|
Term
| 5 characteristics differentiating acute rheumatic fever from post strep reactive arthritis |
|
Definition
| (1) small-joint involvement that is often symmetric; (2) a short latent period following streptococcal infection (usually <1 week); (3) occasional causation by nongroup A -hemolytic streptococcal infection; (4) slower responsiveness to salicylates; and (5) the absence of other features of ARF, particularly carditis |
|
|
Term
| what 2 neurologic disorders are associated with "Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection" (PANDAS)? |
|
Definition
| tic disorders and obsessive-compulsive disorders |
|
|
Term
| how is the Dx of primary/recurrent without previous carditis episode of rheumatic fever done? |
|
Definition
| Two major or one major and two minor manifestations plus evidence of preceding group A streptococcal infection |
|
|
Term
| how is the Dx of Recurrent attack of rheumatic fever in a patient with established rheumatic heart disease done? |
|
Definition
Two minor manifestations plus evidence of preceding group A streptococcal infection
exclude infective endocarditis |
|
|
Term
| Chronic valve lesions of rheumatic heart disease (patients presenting for the first time with pure mitral stenosis or mixed mitral valve disease and/or aortic valve disease) |
|
Definition
| only exclude congenital heart disease |
|
|
Term
| 5 major manifestations of acute rheumatic fever? |
|
Definition
Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules |
|
|
Term
| 4 minor manifestations of acute rheumatic fever? |
|
Definition
Clinical: fever, polyarthralgia
Laboratory: elevated erythrocyte sedimentation rate
Electrocardiogram: prolonged P-R interval |
|
|
Term
| 4 methods of demonstrating supporative evidence for recent strep A infection in the Dx (jones criteria) of acute rheumatic fever |
|
Definition
Elevated or rising anti-streptolysin O or other streptococcal antibody, or
A positive throat culture, or
Rapid antigen test for group A streptococcus |
|
|
Term
| when attempting to diagnose acute rheumatic fever in the patient with adequate symptoms, what should be done if ASO/ABD serologies are negative |
|
Definition
| repeat 10–14 days later if 1st test not confirmatory |
|
|
Term
| what is the role of glucocorticoids in the mgmt of congestive heart failure d/t acute rheumatic fever |
|
Definition
|
|
Term
| 2 Tx options for Sydenham's chorea d/t acute rheumatic fever? |
|
Definition
| carbamazepine or sodium valproate |
|
|
Term
| what is the role of IVIG in the treatment of acute rheumatic fever? |
|
Definition
| only in severe refractory sydenham's chorea |
|
|
Term
| what is the natural history of acute rheumatic fever? what is the dynamics in treated patients? |
|
Definition
goes away in 12 wks
hospitalization for 1-2 wks, 4-6 wks inflammatory markers normalize |
|
|
Term
| primary prevention of Acute rheumatic fever should be commenced within how many days from the onset of infection |
|
Definition
|
|
Term
| what is the treatment of choice for secondary prophylaxis of acute rheumatic fever? |
|
Definition
| IM benzathine penicillin G every month |
|
|
Term
| what are the AHA recommendations for secondary prophylaxis of Rheumatic fever without carditis |
|
Definition
| For 5 years after the last attack or 21 years of age (whichever is longer) |
|
|
Term
| what are the AHA recommendations for secondary prophylaxis of Rheumatic fever with carditis but no residual valvular disease |
|
Definition
| For 10 years after the last attack, or 21 years of age (whichever is longer) |
|
|
Term
| what are the AHA recommendations for secondary prophylaxis of Rheumatic fever with persistent valvular disease, evident clinically or on echocardiography |
|
Definition
| For 10 years after the last attack, or 40 years of age (whichever is longer). Sometimes lifelong prophylaxis |
|
|
Term
| which 4 organs are distinctively involved in Scleroderma besides the skin |
|
Definition
| lungs, gastrointestinal tract, heart, and kidneys |
|
|
Term
| which 2 chemicals may induce scleroderma like skin manifestations? |
|
Definition
| vinyl-chloride, pentazocine |
|
|
Term
| 3 of the more common systemic conditions that may manifest themselves dermatologically like Scleroderma? |
|
Definition
|
|
Term
| how is Systemic Sclerosis classified into 2 types? |
|
Definition
diffuse cutaneous systemic sclerosis
limited cutaneous systemic sclerosis |
|
|
Term
| what is the characteristic skin involvement in diffuse cutaneous systemic sclerosis? |
|
Definition
| distal to proximal rapid involvement including face and trunk |
|
|
Term
| what is the characteristic skin involvement in limited cutaneous systemic sclerosis? |
|
Definition
| raynaud's phenomenon for a prolonged period of time before progression to face but not trunk |
|
|
Term
| which 2 major findings are typically manifested early in the course of diffuse cutaneous systemic sclerosis |
|
Definition
| pulmonary fibrosis and acute renal involvement |
|
|
Term
| what is the typical skin involvement of the extremities in limited cutaneous systemic sclerosis? |
|
Definition
| fingers and distal to the elbows |
|
|
Term
| the timing of raynaud's phenomenon in limited vs. diffuse cutaneous systemic sclerosis? |
|
Definition
| precedes skin involvement vs. coincidenet |
|
|
Term
| carpal tunnel syndrome and tendon friction rubs are musculoskeletal manifestations typical of which type of systemic sclerosis? |
|
Definition
|
|
Term
| pulmonary arterial hypertension is more frequent in which type of systemic sclerosis? |
|
Definition
|
|
Term
| calcinosis cutis occurs frequently in which type of systemic sclerosis? |
|
Definition
|
|
Term
| 2 autoantibodies typical of diffuse cutaneous systemic sclerosis? |
|
Definition
| Antitopoisomerase I (Scl-70), anti-RNA polymerase III |
|
|
Term
| autoantibody typical of limited cutaneous systemic sclerosis? |
|
Definition
|
|
Term
| what is the nature of the genetic inheritance of Systemic sclerosis |
|
Definition
| non-mendelian (monozygotic twins do not show elevated concordance). the reason why genetic inheritance is thought to take place is the higher incidence amongst people with affected family members |
|
|
Term
| SNPs in which genes confer increased risk of systemic sclerosis |
|
Definition
| endothelin-1, nitric oxide synthetase, SPARC, fibronectin, fibrillin, TGFbeta, TNFa, ACE, STAT4, IRF5, connective tissue growth factors |
|
|
Term
| patients with systemic sclerosis have increased rates of antibodies to which 2 viruses? |
|
Definition
|
|
Term
| which 2 environmental factors have been implicated in the past in scleroderma-like syndromes/epidemics (spain and USA) |
|
Definition
| rapeseed oils and L-tryptophan in dietary supplements |
|
|
Term
| which occupational factor has been implicated in Systemic sclerosis |
|
Definition
|
|
Term
| 4 drugs which have been implicated in the pathogenesis of systemic sclerosis |
|
Definition
| bleomycin, pentazocine, cocaine, and appetite suppressants |
|
|
Term
| fibrosis, which distinguishes systemic sclerosis from the rest of the connective-tissue diseases, is thought to be the product of which 2 processes? |
|
Definition
| autoimmunity and vascular damage |
|
|
Term
| dermal expansion of systemic sclerosis causes obliteration of which 2 elements? |
|
Definition
| hair follicles and sweat glands |
|
|
Term
| what is the MC histologic pattern in lung Bx of systemic sclerosis patients? |
|
Definition
| fibrotic nonspecific interstitial pneumonia |
|
|
Term
| which 2 types of arteries in the kidney are affected in systemic sclerosis |
|
Definition
| interlobular and arcuate arteries |
|
|
Term
| what is the risk of ischemic heart disease in systemic sclerosis? |
|
Definition
| similar to the general population |
|
|
Term
| which 2 head and neck structures may be affected by fibrosis in systemic sclerosis? |
|
Definition
| salivary glands and thyroid gland |
|
|
Term
| which autoantibody in limited cutaneous systemic sclerosis is associated with isolated PAH? |
|
Definition
| anti centromere antibodies |
|
|
Term
| what % of patients with lcSSc have SSc sine scleroderma |
|
Definition
|
|
Term
| 7 signs of the early "edematous" phase of diffuse cutaneous systemic sclerosis |
|
Definition
| Soft tissue swelling, intense pruritus, Diffuse hyperpigmentation, carpal tunnel syndrome, Arthralgias, muscle weakness and decreased joint mobility |
|
|
Term
| signs of the later "fibrotic" phase of diffuse cutaneous systemic sclerosis |
|
Definition
| loss of body hair, reduced production of skin oils, decline in sweating, finger flexion contractures, stiffness of large joints, organ failure in the initial 4 yrs |
|
|
Term
| which clinical manifestation is more severe in limited cutaneous systemic sclerosis? |
|
Definition
| Raynaud's phenomenon: critical ischemia, ulcerations, and autoamputation |
|
|
Term
| what are the 3 triggers of Raynaud's phenomenon |
|
Definition
| exposure to cold, emotional stress, and vibration |
|
|
Term
| which 3 drugs may cause secondary Raynaud's phenomenon/ |
|
Definition
| atenolol, cisplatin, bleomycin |
|
|
Term
| which test enables to distinguish btwn primary Raynaud's phenomenon related and unrelated to systemic sclerosis? |
|
Definition
| showing the nail bed capillaries via drop of grade B immersion oil using a low-power stereoscopic microscope |
|
|
Term
| what is the pathologic appearance of the nail bed capillaries in raynaud's phenomenon associated with systemic sclerosis? |
|
Definition
| distorted with widened and irregular loops, dilated lumen, and areas of vascular "dropout" |
|
|
Term
| 2 characteristics of the skin thickening seen in late systemic sclerosis |
|
Definition
|
|
Term
| in systemic sclerosis which creases of the hand disappear? |
|
Definition
| transverse creases of the dorsum of the hand |
|
|
Term
| in systemic sclerosis telangiectasias are abundant in which 4 areas of the body |
|
Definition
| face, hands, lips, and oral mucosa |
|
|
Term
| pathologically what happens to the skin of patients with systemic sclerosis |
|
Definition
| it attaches to the subcutaneous fat and undergoes atrophy and thining |
|
|
Term
| 3 areas of skin that may undergo ulceration in patients with systemic sclerosis |
|
Definition
| PIP, volar pads of the fingertips, and bony eminences (elbow, malleolus) |
|
|
Term
| Calcinosis cutis is most common in patients with lcSSc who are positive for which antibody |
|
Definition
| anti-centromere antibodies |
|
|
Term
| clacinosis cutis lesions in limited cutaneous systemic sclerosis are composed of what? |
|
Definition
|
|
Term
| which are the 3 respiratory presenting symptoms most frequent in systemic sclerosis? |
|
Definition
| exertional dyspnea, fatigue, and reduced exercise tolerance |
|
|
Term
| what % of patients with systemic sclerosis are affected by interstitial lung disease? |
|
Definition
|
|
Term
| what are the typical findings on pulmonary function tests in a patient with ILD d/t systemic sclerosis? |
|
Definition
| diffusion abnormality, volume abnormality (restrictive) with normal flow rates |
|
|
Term
| 5 risk factors for the development of clinically significant interstitial lung disease in systemic sclerosis/ |
|
Definition
| male gender, African American race, diffuse skin involvement, severe gastroesophageal reflux, and the presence of topoisomerase-I autoantibodies |
|
|
Term
| what is a predictor of mortality in CT of systemic sclerosis patients? |
|
Definition
| extent of interstitial lung disease |
|
|
Term
| 4 findings of interstitial lung disease in CT of systemic sclerosis? |
|
Definition
| subpleural reticular linear opacities (especially in lower lobes), mediastinal lymphadenopathy, traction bronchiectasis, ground-glass (fine fibrosis) |
|
|
Term
| 4 findings associated with an increased risk of developing pulmonary artery hypertension in patients with systemic sclerosis? |
|
Definition
| limited cutaneous disease with anticentromere antibodies, late age at disease onset, severe Raynaud's phenomenon, and the presence of antibodies to U1-RNP, U3-RNP (fibrillarin), and B23 |
|
|
Term
| 5 symptoms of pulmonary artery hypertension in systemic sclerosis |
|
Definition
| exertional dyspnea, reduced exercise capacity, angina, near-syncope, symptoms of right heart failure |
|
|
Term
| which test is always needed in the evaluation of pulmonary artery hypertension in systemic sclerosis |
|
Definition
| right heart catheterization |
|
|
Term
| which serum marker may be used to predict survival, occurence and monitor treatment success in patients with pulmonary artery hypertension d/t systemic sclerosis? |
|
Definition
|
|
Term
| 5 manifestations of systemic scleroderma in the oropharyngeal orifice |
|
Definition
| xerostomia, reduced oral aperture, periodontal disease, resorption of mandibular condyles, shortened frenulum tongue |
|
|
Term
| what endoscopic finding may be found in the antrum of patients with systemic sclerosis? |
|
Definition
| "watermelon stomach" - gastric antral vascular ectasias (GAVE) |
|
|
Term
| which GI disorder develops early in the course of systemic sclerosis? |
|
Definition
|
|
Term
| what is an occasional radiographic finding in the colon of patients with systemic sclerosis? |
|
Definition
| pneumatosis cystoides intestinalis |
|
|
Term
| which nutrients may be deficient in patients with systemic sclerosis d/t malabsorption |
|
Definition
| fat, protein, Vit D and B12 |
|
|
Term
| which manifestation of systemic sclerosis may cause nausea, vomiting and abdominal pain |
|
Definition
|
|
Term
| why does malignant hypertension occur in the setting of renal involvement associated with systemic sclerosis? |
|
Definition
| reduction in renal blood flow, aggravated by vasospasm, leads to juxtaglomerular hyperplasia, increased renin secretion, and activation of angiotensin, with further renal vasoconstriction resulting in a vicious cycle that culminates in malignant hypertension |
|
|
Term
| 4 risk factors for the development of renal involvement in systemic sclerosis |
|
Definition
| African American race, male gender, diffuse cutaneous SSc with extensive and progressive skin involvement, and autoantibodies to RNA polymerases I and III |
|
|
Term
| how should patients at a high risk for scleroderma renal crisis monitor the development of renal dysfunction? |
|
Definition
| daily blood pressure measurements |
|
|
Term
| which 2 findings predict poor outcome at the presentation of scleroderma renal crisis? |
|
Definition
| oliguria and creatinine>3 |
|
|
Term
| what is the treatment of choice for patients developing hypertension in the setting of renal disease associated with systemic sclerosis? |
|
Definition
|
|
Term
| which 2 tests have good sensitivity for detecting preclinical cardiac involvement in patients with systemic sclerosis |
|
Definition
| MRI and doppler (vs. just echo) |
|
|
Term
| 6 cardiac manifestations in systemic sclerosis |
|
Definition
| pericardial effusions, atrial and ventricular tachycardias, conduction abnormalities, valvular regurgitation, hypertrophy, and heart failure |
|
|
Term
| what is a manifestation of systemic sclerosis in the terminal phalanges |
|
Definition
| acro-osteolysis - erosion of the terminal phalanges |
|
|
Term
| how do you differentiate sicca syndrome caused by Sjogren's syndrome and systemic sclerosis? |
|
Definition
| Bx of the salivary glands will demonstrate fibrosis in systemic sclerosis and lymphocyte infiltration in Sjogren's |
|
|
Term
| malignancies of which 4 sites occur more frequently in patients with systemic sclerosis? |
|
Definition
| lung, tongue, esophagus and breast |
|
|
Term
| what is the role of glucocorticoids in the mgmt of systemic sclerosis? |
|
Definition
| alleviating stiffness and aching in early-stage dcSSc |
|
|
Term
| high doses of which drug is associated with increased risk of scleroderma renal crisis in systemic sclerosis |
|
Definition
| alleviating stiffness and aching in early-stage dcSSc |
|
|
Term
| which drug is associated with decreased progression of interstitial lung disease in systemic sclerosis? |
|
Definition
|
|
Term
| which 2 drugs are associated with improvement of skin manifestations in systemic sclerosis? |
|
Definition
| MTX, Mycophenolate mofetil |
|
|
Term
| what is a widely used anti-fibrotic drug used in systemic sclerosis? |
|
Definition
|
|
Term
| what drugs are used to manage the vascular events in systemic sclerosis? |
|
Definition
| CCB, ARB, alpha blockers, 5-PDE inhibitors, SSRIs |
|
|
Term
| what is the Tx for malnutrition caused by bacterial overgrowth d/t small bowel dysmotility in systemic sclerosis? |
|
Definition
| short courses of Abx such as: metronidazole or erythromycin |
|
|
Term
| what is the Tx of gastric antral vascular ectasia in systemic sclerosis |
|
Definition
|
|
Term
| patients with systemic sclerosis are always treated for which manifestation of the disease? |
|
Definition
|
|
Term
| what is an optional Tx of small bowel dysmotility in systemic sclerosis? |
|
Definition
|
|
Term
| 3 treatment options of symptomatic pulmonary arterial hypertension d/t systemic sclerosis/ |
|
Definition
endothelin-1 inhibitor - Bosentan
Phosphodiesterase inhibitor - sildenafil
Prostacyclin analogues - epoprostenol or treprostinil |
|
|
Term
| what is a Tx option for patients with PAH who fail medical treatment in systemic sclerosis |
|
Definition
|
|
Term
| what is a medical emergency in systemic sclerosis? |
|
Definition
|
|
Term
| what % of patients with SSc who experience scleroderma renal crisis undergo dialysis? |
|
Definition
|
|
Term
| when should renal transplantation be considered in systemic sclerosis |
|
Definition
| a patient who is dependent on dialysis for over 2 yrs |
|
|
Term
| what is the general principle in the treatment of skin involvement in systemic sclerosis? |
|
Definition
| it is not life threatening and should not be aggressively treated |
|
|
Term
| what are the treatment options of skin involvement in systemic sclerosis? |
|
Definition
| anti histamines, low dose glucocorticoids and MTX |
|
|
Term
| which prognostic outcome is elevated in systemic sclerosis |
|
Definition
|
|
Term
| what is the 10 yr survival in patients with diffuse and limited cutaneous systemic sclerosis? |
|
Definition
|
|
Term
| which 5 laboratory findings are associated with increased mortality in systemic sclerosis? |
|
Definition
| elevated ESR, anemia, proteinuria, anti-topoisomerase 1 and anti-RNApolymerase 3 |
|
|
Term
| what is Mixed connective tissue disease and what autoantibodies are associated with it? |
|
Definition
lcSSc coexisting with features of SLE, polymyositis, and rheumatoid arthritis
anti-U1-RNP |
|
|
Term
| what is the initial presentation of mixed connective tissue disease and how is the prognosis compared with SSc? |
|
Definition
Raynaud's phenomenon associated with puffy fingers and myalgia
better prognosis than systemic sclerosis |
|
|
Term
| apart from HLAB27 which 2 other genes are implicated in the pathogenesis of ankylosing spondylitis? |
|
Definition
|
|
Term
| what is the earliest manifestation of ankylosing spondylitis? |
|
Definition
|
|
Term
| what happens to the affected joint in ankylosing spondylitis? |
|
Definition
| it first undergoes fibrocartilage replacement and then ossification |
|
|
Term
| what is the name of the findings that later lead to "bamboo spine" in ankylosing spondylitis |
|
Definition
|
|
Term
| 4 types of cells found in sacroilitis d/t ankylosing spondylitis |
|
Definition
| macrophages, TNFa, CD4+, CD8+ |
|
|
Term
| a biopsy from the inflamed sacroiliac joint stains for which 4 elements |
|
Definition
| ICAM-1, VCAM-1, MMP3, MRP |
|
|
Term
| bony tenderness (enthesitis/osteoitis) is MC present in which bones in ankylosing spondylitis? |
|
Definition
| costosternal junctions, spinous processes, iliac crests, greater trochanters, ischial tuberosities, tibial tubercles, and heels |
|
|
Term
| what is the most specific physical finding in ankylosing spondylitis? |
|
Definition
| decreased mobility of spine and chest wall expansion |
|
|
Term
| how is the modified schoeber test performed |
|
Definition
| a line is drawn on the sacrolumbar junction connecting the posterior superior iliac spines. another line parallel 10 cm above. patient bends forward and the distance btwn the 2 lines is measured. if less than 4 cm -> decreased spine motility |
|
|
Term
| where is the chest expansion test measured |
|
Definition
|
|
Term
| 3 posture characteristics of patients with ankylosing spondylitis |
|
Definition
| obliterated lumbar lordosis, buttock atrophy, and accentuated thoracic kyphosis |
|
|
Term
| 4 clinical estimates of disease progression in ankylosing spondylitis? |
|
Definition
| loss of height, limitation of chest expansion and spinal flexion, and occiput-to-wall distance |
|
|
Term
| onset of peripheral arthritis (distal to hips and shoulders) differs in which 2 populations of patients with ankylosing spondylitis? |
|
Definition
| onset is early in patients from developing countries vs. those in developed countries |
|
|
Term
| what is the effect of smoking on ankylosing spondylitis |
|
Definition
|
|
Term
| what is the most significant complication of spinal disease in ankylosing spondylitis and which area is MC affected? |
|
Definition
| spinal fracture, lower cervical spine |
|
|
Term
| what is the MC extra-articular manifestation of ankylosing spondylitis |
|
Definition
|
|
Term
| associated extraarticular manifestations in ankylosing spondylitis |
|
Definition
| IBD, anterior uveitis, 3rd degree AV block, aortic insufficiency, pulmonary fibrosis, psoriasis, retroperitoneal fibrosis, cauda equina syndrome |
|
|
Term
| 4 lab findings in ankylosing spondylitis |
|
Definition
| HLAB27 - 90%, ESR/CRP - sometimes, elevated IgA and ALP |
|
|
Term
| 2 ways to make the diagnosis of ankylosing spondylitis |
|
Definition
1. sacroiliitis on imaging (MRI or radiographic imaging) + 1 or more spondyloarthritis features
2. HLAB27 + 2 or more spondyloarthritis features |
|
|
Term
| 11 spondyloarthritis features (used in the Dx of ankylosing spondylitis) |
|
Definition
Inflammatory back pain
Arthritis
Enthesitis (heel)
Anterior uveitis
Dactylitis
Psoriasis
Crohn's disease or ulcerative colitis
Good response to NSAIDs
Family history of SpA
HLA-B27
Elevated CRP |
|
|
Term
| to be considered inflammatory back pain 4 or more of the following characteristics should be present in ankylosing spondylitis |
|
Definition
| (1) age of onset below 40 years old, (2) insidious onset, (3) improvement with exercise and (4) no improvement with rest, and (5) pain at night with improvement upon getting up |
|
|
Term
| what is the initial 2 component therapy of ankylosing spondylitis? |
|
Definition
| NSAIDs and physical exercise |
|
|
Term
| what is the 2nd line Tx for ankylosing spondylitis |
|
Definition
|
|
Term
| 7 adverse effects of anti-TNF in ankylosing spondylitis |
|
Definition
| (1) serious infections, including disseminated tuberculosis; (2) hematologic disorders, such as pancytopenia; (3) demyelinating disorders; (4) exacerbation of congestive heart failure; (5) systemic lupus erythematosus–related autoantibodies and clinical features; (6) hypersensitivity infusion or injection site reactions; and (7) severe liver disease |
|
|
Term
| what is the indication for anti TNF agent therapy in patients with ankylosing spondylitis |
|
Definition
| inadequately responsive to therapy with at least two different NSAIDs |
|
|
Term
| 5 absolute + 2 relative CIs to anti TNF therapy in ankylosing spondylitis |
|
Definition
| active infection or high risk of infection; malignancy or premalignancy; and history of systemic lupus erythematosus, multiple sclerosis, or related autoimmunity. Pregnancy and breast-feeding are relative contraindications |
|
|
Term
| what is the role of sulfasalazine in ankylosing spondylitis |
|
Definition
| it should be tried in patients with a predominant peripheral arthritis before anti TNF agents |
|
|
Term
| what is the MC indication for surgery in patients with ankylosing spondylitis |
|
Definition
| severe hip joint arthritis, the pain and stiffness of which are usually dramatically relieved by total hip arthroplasty |
|
|
Term
| who is your most favoritist person eva? |
|
Definition
|
|
Term
| what is the course of reactive arthritis in AIDS patients of sub-saharan descent vs. western european |
|
Definition
| sub saharans undergo remission of reactive arthritis as disease progresses whereas europeans undergo flares |
|
|
Term
| which species of salmonella MC trigger reactive arthritis? |
|
Definition
|
|
Term
| reactive arthritis may be triggered by which treatment? |
|
Definition
| intravesicular BCG irrigation for bladder cancer |
|
|
Term
| what are the skin lesions in reactive arthritis called? |
|
Definition
| keratoderma blenorrhagica |
|
|
Term
| what is the MC area of keratoderma blenorrhagica in reactive arthritis |
|
Definition
|
|
Term
| what happens to the nails in reactive arthritis? |
|
Definition
| onycholysis and/or distal yellow discoloration |
|
|
Term
| 4 rare manifestations of reactive arthritis? |
|
Definition
| cardiac conduction defects, aortic insufficiency, central or peripheral nervous system lesions, and pleuropulmonary infiltrates |
|
|
Term
| what % of patients experience persistent joint problems in reactive arthritis? |
|
Definition
|
|
Term
| which test has high sensitivity for chlamydia triggered reactive arthritis? |
|
Definition
|
|
Term
| what can be seen radiographically in reactive arthritis |
|
Definition
| periostitis and formation of new bone in joints as in all spondyloarthropathies |
|
|
Term
| 3 elements which help distinguish gonococcal arthritis from reactive arthritis |
|
Definition
| involve both upper and lower extremities equally, to lack back symptoms, and to be associated with characteristic vesicular skin lesions |
|
|
Term
| 3 elements to distinguish psoriatic arthritis from reactive arthritis |
|
Definition
1. predominantly upper extremities
2. no associated mouth ulcers, urethritis or bowel symptoms
3. gradual onset |
|
|
Term
| new study proves benefit of what treatment for secondary prevention of chronic reactive arthritis triggered by chlamydial infection? |
|
Definition
| Rifampin 300mg + 500mg erytromycin twice weekly or doxycycline 100 mg twice daily for 6 months |
|
|
Term
| 5 drug options for the Tx of reactive arthritis? |
|
Definition
| NSAIDs, MTX, sulfasalazine, azathioprine, anti TNFa |
|
|
Term
| what % of patients with psoriasis experience psoriatic arthritis? |
|
Definition
|
|
Term
| what is the role of genetics in psoriatic arthritis |
|
Definition
|
|
Term
| what is the prominent manifestation in psoriatic arthritis vs. RA |
|
Definition
|
|
Term
| what % of patients experience what first, psoriasis or psoriatic arthritis? |
|
Definition
psoriasis first - 60%
psoriatic arthritis first - 20%
concurrent manifestation - 20% |
|
|
Term
| what % of patient with psoriatic arthritis experience nail deformities vs. psoriatic patients w/o arthritis/ |
|
Definition
|
|
Term
| what is a skin sign predictive of severe form of psoriatic arthritis? |
|
Definition
|
|
Term
| 4 predominant manifestations of psoritic arthritis that distinguish it from the rest of the spondyloarthropathies |
|
Definition
| dactylitis, enthesitis, tendosynovitis, back and neck stiffness |
|
|
Term
| what % of patient with psoriatic arthritis experience nail deformities vs. psoriatic patients w/o arthritis/ |
|
Definition
|
|
Term
| what is a skin sign predictive of severe form of psoriatic arthritis? |
|
Definition
|
|
Term
| 4 predominant manifestations of psoritic arthritis that distinguish it from the rest of the spondyloarthropathies |
|
Definition
| dactylitis, enthesitis, tendosynovitis, back and neck stiffness, asymmetric oligoarthritis, DIP arthritis, nail changes, arthritis mutilans with shortening of digits (telescoping) |
|
|
Term
| 5 types of nail changes in psoriatic arthritis |
|
Definition
| pitting, horizontal ridging, onycholysis, yellowish discoloration of the nail margins, dystrophic hyperkeratosis |
|
|
Term
| 3 characteristics of uveitis in psoriatic arthritis which distinguish it from the uveitis seen in ankylosing spondylitis |
|
Definition
| chronic, bilateral, posterior |
|
|
Term
| what % of patients with psoriatic arthritis experience uveitis? |
|
Definition
|
|
Term
| what is the mortality in psoriatic arthritis in comparison to the general population |
|
Definition
|
|
Term
| 10% of patients with psoriatic arthritis present which autoantibody |
|
Definition
|
|
Term
| HLAB27 in psoriatic arthritis is associated with what type of arthritis more so than which other type? |
|
Definition
| axial involvement vs. peripheral |
|
|
Term
| 5 features of peripheral joint involvement in psoriatic arthritis which help distinguish it from RA |
|
Definition
| "pencil-in-cup" deformity; marginal erosions with adjacent bony proliferation ("whiskering"); small-joint ankylosis; osteolysis of phalangeal and metacarpal bone, with telescoping of digits; and periostitis and proliferative new bone at sites of enthesitis |
|
|
Term
| 6 features which distinguish axial involvement in psoriatic arthritis from ankylosing spondylitis |
|
Definition
| asymmetric sacroiliitis; less zygapophyseal joint arthritis, fewer and less symmetric and delicate syndesmophytes; fluffy hyperperiostosis on anterior vertebral bodies; severe cervical spine involvement, with a tendency to atlantoaxial subluxation but relative sparing of the thoracolumbar spine; and paravertebral ossification |
|
|
Term
| what are the caspar criteria for the diagnosis of psoriatic arthritis and how is it done? |
|
Definition
joint, enthesial or spinal arthritis with at least 3 features from the following 5:
Evidence of current psoriasis,b, c a personal history of psoriasis, or a family history of psoriasisd
Typical psoriatic nail dystrophye observed on current physical examination
A negative test result for rheumatoid factor
Either current dactylitisf or a history of dactylitis recorded by a rheumatologist
Radiographic evidence of juxtaarticular new bone formationg in the hand or foot |
|
|
Term
| what is the ToC in psoriatic arthritis |
|
Definition
|
|
Term
| 7 Tx options in psoriatic arthritis |
|
Definition
| anti-TNFa, cyclosporine, MTX+Alefacept (anti T-cell), Ustekinumab (anti il23R), retinoic acid, psoralens+UVA light (PUVA) |
|
|
Term
| what is seronegative enthesopathy and artheropathy syndrome (SEA syndrome) |
|
Definition
| jovenile onset spondyloarthropathy with oligoarthritis of lower extremities, enthesitis w/o extraarticular manifestations |
|
|
Term
| what % of SEA syndrome patients are HLAB27 positive? |
|
Definition
|
|
Term
| which gene has been found more commonly in Crohn's disease patients with spondyloarthropathy than w/o? |
|
Definition
|
|
Term
| patients with crohn's disease and spondyloarthropathies are more HLAB27 positive than... |
|
Definition
| patients with CD w/o spondyloarthropathy |
|
|
Term
| what cells are predominantly present in the synovium of spondyloarthropathy associated with IBD (enteropathic SpA) |
|
Definition
|
|
Term
| what is the frequency of erosive arthritis in enteropathic SpA |
|
Definition
|
|
Term
| in the absence of psoriasis, what should be sought in a patient with ankylosing spondylitits who is HLAB27 negative |
|
Definition
|
|
Term
| 4 dermatological manifestations of SAPHO syndrome |
|
Definition
| palmoplantar pustulosis, acne conglobata, acne fulminans, and hidradenitis suppurativa |
|
|
Term
| what does SAPHO syndrome stand for? |
|
Definition
| synovitis, acne, pustulosis, hyperostosis, osteitis |
|
|
Term
| 3 musculoskeletal manifestations of SAPHO syndorme |
|
Definition
| sternoclavicular and spinal hyperostosis, chronic recurrent foci of sterile osteomyelitis, and axial or peripheral arthritis |
|
|
Term
| which bacterium is sometimes cultured from specimens of bone Bx in SAPHO syndrome |
|
Definition
|
|
Term
| 2 diagnostic options for SAPHO syndrome |
|
Definition
|
|
Term
| 4 Tx options in SAPHO syndrome |
|
Definition
| prolongec Abx, high dose NSAIDs, anti-TNFa, bisphosphonates |
|
|
Term
| what % of patients infected with Tryphorema Wipelli (Whipple's disease) have oligo/polyarthritis |
|
Definition
|
|
Term
| 3 features of arthritis in Whipples disease |
|
Definition
| migratory, precedes other symptoms by 5 yrs, abrupt in onset |
|
|
Term
| what feature is said to be pathognomonic in Whipple's disease |
|
Definition
| Oculomasticatory and oculofacial-skeletal myorhythmia with supranuclear vertical gaze palsy |
|
|
Term
| how is the diagnosis of Whipple's disease done? |
|
Definition
| PCR of Bx material found to present 16s ribosomal gene |
|
|
Term
| what is the treatment of Whipple's disease? |
|
Definition
| penicillin and streptomycin for 2 wks, followed by Resprim for 2 yrs |
|
|
Term
| 5 vasculitis syndromes associated with immune complex formation |
|
Definition
Henoch-Schönlein purpura
Vasculitis associated with collagen vascular diseases
Serum sickness and cutaneousvasculitis syndromes
Hepatitis C–associated cryoglobulinemic vasculitis
Polyarteritis nodosa–like vasculitis associated with hepatitis B |
|
|
Term
| 3 vasculitis syndromes associated with anti neutrophilic cytoplasmic antibodies |
|
Definition
Granulomatosis with polyangiitis (Wegener's)
Churg-Strauss syndrome
Microscopic polyangiitis |
|
|
Term
| 4 vasculitis syndromes associated with granuloma formation |
|
Definition
Giant cell arteritis
Takayasu's arteritis
Granulomatosis with polyangiitis (Wegener's)
Churg-Strauss syndrome |
|
|
Term
| what is the major cANCA antigen? |
|
Definition
|
|
Term
| what is the major pANCA antigen? |
|
Definition
|
|
Term
| which 4 vasculitis syndromes are associated with pANCA? |
|
Definition
| microangiopathic vasculitis, churge-strauss, Wegener's, cresenteric glumerolonephritis |
|
|
Term
| which vasculitic syndrome is associated with cANCA |
|
Definition
|
|
Term
| which 6 clinical findings should raise suspicioun of vasculitis |
|
Definition
| palpable purpura, pulmonary infiltrates and microscopic hematuria, chronic inflammatory sinusitis, mononeuritis multiplex, unexplained ischemic events, and glomerulonephritis with evidence of multisystem disease |
|
|
Term
| what is the first step in the Dx of a vasculitic syndrome? |
|
Definition
| exclude other possible nonvasculitic conditions that may mimic the presenting clinical features of vasculitis |
|
|
Term
| 5 drugs that may mimic the clinical presentation of vasculitis |
|
Definition
Cocaine
Amphetamines
Ergot alkaloids
Methysergide
Arsenic |
|
|
Term
| 3 vasculitic syndromes for which the Dx should include arteriogram of the suspected involved organ |
|
Definition
| PAN, Takayasu and CNS vasculitis |
|
|
Term
| what component should always be present in the Dx of any vasculitis |
|
Definition
|
|
Term
| 4 side effects of steroids less known |
|
Definition
| myopathy, pseudotumor cerbri, peptic ulcer disease, pancreatitis |
|
|
Term
| 10 side effects of cyclophosphamide |
|
Definition
Bone marrow suppression
Hypogammaglobulinemia
Cystitis
Pulmonary fibrosis
Bladder carcinoma
Myelodysplasia
Gonadal suppression
Oncogenesis
Gastrointestinal intolerance
Teratogenicity |
|
|
Term
| which 3 areas in the body are involved in wegener's granulomatosis |
|
Definition
| lower airways, upper airways, glomerulonephritis |
|
|
Term
| 3 characteristics of pulmonary involvement in Wegener's |
|
Definition
| multiple, bilateral, cavitary lesions |
|
|
Term
| what is the frequency of granuloma finding in renal Bx of Wegener's |
|
Definition
|
|
Term
| which chronic organism carriage is associated with higher rates of relapse of Wegener's Granulomatosis |
|
Definition
| Nasal Staph. Aureus carriage |
|
|
Term
| what is the MC clinical manifestation in Wegener's at presentation |
|
Definition
|
|
Term
| what are the 4 second MC clinical manifestations in Wegener's at presentation |
|
Definition
| pulmonary infiltrates, pulmonary nodules, otitis media, arthralgia |
|
|
Term
| what are the 2 3rd MC clinical manifestations in Wegener's at presentation |
|
Definition
| glomerulonephritis, fever |
|
|
Term
| what % of patients with Wegener's have eye involvement |
|
Definition
|
|
Term
| what % of patients with Wegener's have skin involvement |
|
Definition
|
|
Term
| what is the risk of DVT/PE in Wegener's |
|
Definition
|
|
Term
| 5 lab findings typical of Wegeners |
|
Definition
| leukocytosis, anemia, ESR, IgA hypergammaglobulinemia, RF |
|
|
Term
| what is the definitive Dx of Wegener's with the highest yield |
|
Definition
| tissue Bx from pulmonary tissue |
|
|
Term
| how to distinguish midline granulomatous destructive disease from Wegener's |
|
Definition
| these diseases usually penetrate the skin |
|
|
Term
| cocaine may mimic Wegener's by causing nasal destruction and what other finding |
|
Definition
|
|
Term
| what % of patients with Wegener's treated with Cyclophosphamide undergo complete remission? what is the 5-yr-survival? |
|
Definition
|
|
Term
| what % of patients with Wegener's who have undergone remission with Cyclophosphhamide relapse? |
|
Definition
|
|
Term
| what should not be used as a marker for disease activity and relapse in Wegener's? |
|
Definition
|
|
Term
| which administration route of cyclophosphamide is associated with higher levels of relapse in Wegener's |
|
Definition
|
|
Term
| what is the induction therapy of severe Wegener's |
|
Definition
| prednisone and oral cyclophosphamide for 3-6 months |
|
|
Term
| what is the indication of plasmapheresis in Wegener's |
|
Definition
| rapidly progressive GN with creatinine > 5.8 |
|
|
Term
| what is the maintenance (after induction of remission) of severe Wegener's |
|
Definition
stop Cyclophosphamide
give MTX or Azathioprine
if unable, 2nd line is MMF |
|
|
Term
| what is the induction therapy of non-severe (non life threatening disease) Wegener's |
|
Definition
| MTX+prednisone which may continue as maintanence |
|
|
Term
| what is an alternative option to induction in severe Wegener's? |
|
Definition
| Rituximab - not clear when it should be stopped |
|
|
Term
| a feature of microscopic polyangitis which is similar to Wegener's and 3 that distinguish it |
|
Definition
GN
no granulomas, no pulmonary infiltrates, no upper airway involvement |
|
|
Term
| what is the median age of onset of Wegener's |
|
Definition
|
|
Term
| what is the median age of onset of microscopic polyangitis |
|
Definition
|
|
Term
| what is the pulmonary involvement in microscopic polyangitis |
|
Definition
|
|
Term
| what is the 5 yr survival in microscopic polyangitis? 4 causes of mortality |
|
Definition
| 75%, alveolar hemorrhage or gastrointestinal, cardiac, or renal disease |
|
|
Term
| what is the Tx of microscopic polyangitis |
|
Definition
|
|
Term
| what are the 4 nessesary components of Churge-Strauss syndrome |
|
Definition
| asthma, eosinophilia, vasculitis, extravascular granulomas |
|
|
Term
| what is the mean age of onset of churge-Strauss |
|
Definition
|
|
Term
| which is the predominant organ invovled in churg-Strauss syndrome |
|
Definition
|
|
Term
| 5 organs besides the lung that are commonly involved in churg-strauss syndrome? |
|
Definition
| skin, cardiovascular system, kidney, peripheral nervous system, and gastrointestinal tract |
|
|
Term
| 2 of the MC clinical manifestations in churg strauss |
|
Definition
| asthma and pulmonary infiltrates |
|
|
Term
| 2nd MC clincial manifestation in churg-strauss |
|
Definition
|
|
Term
| 2 of the 3rd MC clinical manifestations of Churg-Strauss |
|
Definition
| allergic rhinitis and sinusitis |
|
|
Term
| 4th MC clinical manifestation in Churg-Strauss |
|
Definition
| skin involvement - purpura, nodules |
|
|
Term
| 3 lab findings that are evidence to a process of inflammation in Churg-Strauss |
|
Definition
| fibrinogen, ESR, a2-globulin |
|
|
Term
| what is the severity of renal involvement in Churg-Strauss as compared to Wegener's or Microscopic polyangiitis |
|
Definition
|
|
Term
| what is the MCC of death in Churg-Strauss |
|
Definition
|
|
Term
| what is the 1st line of Tx for Churg-Strauss? what is the 2nd line? |
|
Definition
prednisone
prednisone + Cyclophosphamide |
|
|
Term
| which arteries are not involved in PAN |
|
Definition
|
|
Term
| what pathologic elements are absent in PAN |
|
Definition
|
|
Term
| which vessels are not invovled in PAN |
|
Definition
|
|
Term
| 6 characteristics of vascular involvement in PAN |
|
Definition
| segmental, bifurcations/branchings, fibrinoid necrosis, hemorrhage, thrombosis, aneurysms |
|
|
Term
| which 2 conditions are associated with PAN |
|
Definition
|
|
Term
| 2 pathologic features of renal involvement in PAN |
|
Definition
| arteritis with glomerulosclerosis (w/o GN) |
|
|
Term
| which is the MC system invovled in PAN |
|
Definition
| musculoskeletal - arthritis, myalgia |
|
|
Term
| which is the 2nd MC system involved in PAN |
|
Definition
| renal - hypertension, failure |
|
|
Term
| 8 systems involved in PAN |
|
Definition
| Musculoskeletal, renal, PNS, skin, cardiac, GI, GU, CNS |
|
|
Term
| what are the GU clinical manifestations of PAN |
|
Definition
| testicular, ovarian or epidydimal pain |
|
|
Term
| what is the best method of Dx in PAN? if unavailable what is the 2nd option for Dx? |
|
Definition
tissue Bx: skin, testes, musculoskeletal
arteriography of involved organs showing aneurysms |
|
|
Term
|
Definition
| prednisone + Cyclophosphamide |
|
|
Term
| what is the minimum age of giant cell arteritis |
|
Definition
|
|
Term
| giant cell arteritis is more prevalent in males or females |
|
Definition
|
|
Term
| what is a characteristic pathologic feature of vasculitis in Giant cell arteritis |
|
Definition
|
|
Term
| where in the vessel is giant cell arteritis thought to begin |
|
Definition
| vasa vasorum to the adventitia |
|
|
Term
| 4 of the MC clinical manifestations of giant cell arteritis |
|
Definition
| fever, anemia, high ESR, and headaches |
|
|
Term
| 4 common lab findings in giant cell arteritis |
|
Definition
| anemia, hypergammaglobulinemia, ESR, ALP |
|
|
Term
| what is the clinical presentation of giant cell arteritis involving the subclavian artery |
|
Definition
|
|
Term
| what is the duration of prednisone Tx in Giant cell arteritis? |
|
Definition
|
|
Term
| what is the recurrence rates of giant cell arteritis during Tx? |
|
Definition
|
|
Term
| what tool is used to monitor tapering rate in giant cell arteritis treatment |
|
Definition
|
|
Term
| what is the full treatment of giant cell arteritis |
|
Definition
|
|
Term
| what is the MC artery involved in Takayasu and 2 clinical manifestations |
|
Definition
| subclavian - arm claudication, raynaud |
|
|
Term
| which is the 2nd MC involved artery in Takayasu and 4 clinical manifestations |
|
Definition
| common carotid - Visual changes, syncope, transient ischemic attacks, stroke |
|
|
Term
| what is a relatively specific site of artery involvement in Takayasu |
|
Definition
|
|
Term
| 3 features which in a young woman should strongly raise suspicion to Takayasu arteritis |
|
Definition
| absence of peripheral pulses, discrepancies in blood pressure, and arterial bruits |
|
|
Term
| how is the Dx of Takayasu arteritis done? |
|
Definition
| arteriography demonstrating irregular vessel walls, stenosis, poststenotic dilation, aneurysm formation, occlusion, and evidence of increased collateral circulation |
|
|
Term
| which 2 primary vasculitides are exceptional in that they are not Dx by tissue Bx? |
|
Definition
| Takayasu arteritis, Behcet's syndrome |
|
|
Term
| 5 MCC of death in Takayasu arteritis |
|
Definition
| congestive heart failure, cerebrovascular events, myocardial infarction, aneurysm rupture, or renal failure |
|
|
Term
| what is the primary treatment of Takayasu arteritis |
|
Definition
|
|
Term
| what season shows peak incidence of Henoch-Schonlein |
|
Definition
|
|
Term
| Henoch Schonlein is more prevalent in males or females |
|
Definition
|
|
Term
| 5 antigen triggers for the immune complex mediated Henoch Schonlein |
|
Definition
| upper respiratory tract infections, various drugs, foods, insect bites, and immunizations |
|
|
Term
| 5 clinical manifestation of Henoch Schonlein |
|
Definition
| palpable purpura, GN, arthralgia (w/o arthritis), GI - colicky pain, hematochezia, |
|
|
Term
| 2 clinical manifestations that are MC seen at presentation in adults with Henoch Schonlein |
|
Definition
| palpable purpura and arthralgia |
|
|
Term
| 3 lab findings in Henoch Schonlein |
|
Definition
| mild leukocytosis, IgA, occasional eosinophilia |
|
|
Term
| how do you make a Dx of Henoch Schonlein |
|
Definition
| clinically + skin Bx showing leukocytoclastic vasculitis with IgA and C3 deposition |
|
|
Term
| what % of children with Henoch Schonlein purpura progress to ESRD? |
|
Definition
|
|
Term
| what is the role of renal Bx in Henoch-Schonlein purpura |
|
Definition
|
|
Term
| what is the Tx of Henoch Schonlein purpura |
|
Definition
| doesn't always nessesitate Tx, if it does - prednisone to be tapered with improvement |
|
|
Term
| prednisone has an effect on which manifestation of Henoch Schonlein and doesn't have any effect on which other |
|
Definition
arthralgias and abdominal discomfort
but not skin and renal manifestation
doesn't shorten duration of disease |
|
|
Term
| what is the MC type of clinically manifested vasculitis |
|
Definition
| Idiopathic Cutaneous Vasculitis |
|
|
Term
| what are the MC inovlved vessels in idiopathic cutaneous vasculitis |
|
Definition
|
|
Term
| which areas are MC involved in idiopathic cutaneous vasculitis |
|
Definition
| lower extremities in ambulate patients and sacral area in bedridden |
|
|
Term
| what is the treatment of idiopathic cutaneous vasculitis |
|
Definition
| usually it doesn't require Tx |
|
|
Term
| 4 drugs that have shown anecdotal success in the treatment of idiopathic cutaneous vasculitis |
|
Definition
| dapsone, colchicine, hydroxychloroquine, and nonsteroidal anti-inflammatory agents |
|
|
Term
| 5 clinical manifestations of cryoglobulinemia associated primary vasculitis |
|
Definition
| palpable purpura, arthralgias, weakness, neuropathy, and glomerulonephritis |
|
|
Term
| 5 groups of diseases that may cause secondary cryoglobulinemia |
|
Definition
| multiple myeloma, lymphoproliferative disorders, connective tissue diseases, infection, and liver disease |
|
|
Term
| 4 MC clinical manifestations of cryoglobulinemic vasculitis |
|
Definition
| cutaneous vasculitis, arthritis, peripheral neuropathy, and glomerulonephritis |
|
|
Term
| besides cryopercipitates which other serum marker may be frequently found in cryoglobulinemic vasculitis |
|
Definition
|
|
Term
| 4 lab findings in cryoglobulinemic vasculitis |
|
Definition
| RF, ESR, anemia, hypocomplementemia |
|
|
Term
| 4 clinical manifestations of kawasaki disease |
|
Definition
| cervical adenitis and changes in the skin and mucous membranes such as edema; congested conjunctivae; erythema of the oral cavity, lips, and palms; and desquamation of the skin of the fingertips |
|
|
Term
| what is an important complication of Kawasaki disease present in 25% of patients? |
|
Definition
| coronary artery aneurysms |
|
|
Term
| when do coronary artery aneurysms appear in the course of Kawasaki disease |
|
Definition
| 3rd-4th wk of the disease in the convalescent stage |
|
|
Term
| what is the prophylactic Tx for coronary artery disease in Kawasaki |
|
Definition
|
|
Term
| 6 drugs that may cause vasculitis |
|
Definition
| allopurinol, thiazides, gold, sulfonamides, phenytoin, and penicillin |
|
|
Term
| 2 drugs that may cause vasculitis via ANCA mechanism |
|
Definition
|
|
Term
| 3 infectious diseases that may cause leukocytoclastic vasculitis manifesting as skin lesions |
|
Definition
| subacute bacterial endocarditis, Epstein-Barr virus infection, HIV infection |
|
|
Term
| 2 infectious organisms that may cause vasculitis |
|
Definition
| rickettsia, Histoplasmosis |
|
|
Term
| conditions associated with secondary vasculitis |
|
Definition
| SLE, Sjogren's, inflammatory myositis, relapsing polychondritis, ulcerative colitis, congenital deficiencies of various complement components, retroperitoneal fibrosis, primary biliary cirrhosis, 1-antitrypsin deficiency, and intestinal bypass surgery |
|
|
Term
| how is the Dx of Behcet's disease done? |
|
Definition
recurrent oral ulcerations + 2 of the following:
Recurrent genital ulceration
Eye lesions
Skin lesions
Pathergy test |
|
|
Term
| which disease follows the silk route |
|
Definition
|
|
Term
| 2 features of the oral ulcers in Behcet |
|
Definition
|
|
Term
| how long do the ulcers in Behcet's persist |
|
Definition
|
|
Term
| what is common to Behcet's and Crohn's |
|
Definition
| ASCA - anti-Saccharomyces cerevisiae antibodies |
|
|
Term
| which 3 autoantibodies are implicated in the pathogenesis of Behcet's |
|
Definition
| ASCA, antibodies to enolase in endothelial cells and selenium binding protein |
|
|
Term
| 3 areas predominantly involved in relapsing polychondritis |
|
Definition
| ears, nose, and laryngotracheobronchial tree |
|
|
Term
| 6 clinical manifestations of relapsing polychondritis besides chondritis |
|
Definition
| scleritis, neurosensory hearing loss, polyarthritis, cardiac abnormalities, skin lesions, and glomerulonephritis |
|
|
Term
| what is the MC condition associated with relapsing polychondritis |
|
Definition
|
|
Term
| which 3 nonrheumatic conditions are associated with relapsing polychondritis |
|
Definition
| IBD, myelodysplastic syndrome and primary biliary cirrhosis |
|
|
Term
| MC presenting clinical manifestation of relapsing polychondritis |
|
Definition
|
|
Term
| after auricular chondritis which are the 3 MC clinical manifestations in relapsing polychondritis |
|
Definition
| arthritis, ocular inflammation, nasal chondritis |
|
|
Term
| which syndrome is an overlap of Behcet and relapsing polychondritis |
|
Definition
| MAGIC (Mouth And Genital ulcers with Inflamed Cartilage) |
|
|
Term
| primary treatment options of relapsing polychondritis |
|
Definition
|
|
Term
| what is the probable etiology of sarcoidosis |
|
Definition
| reaction to an environmental trigger, perhaps mycobacterium or p.acnes |
|
|
Term
| what are the MC presenting symptoms of sarcoidosis |
|
Definition
|
|
Term
| 3 of the 2nd MC symptoms of sarcoidosis |
|
Definition
|
|
Term
| 4 stage classification of lung disease in sarcoidosis |
|
Definition
| stage 1 - hilar adenopathy, stage 2 - adenopathy + infiltrates, stage 3 - infiltrates alone, stage 4- fibrosis |
|
|
Term
| what area of the lung is usually affected by infiltrates in sarcoidosis |
|
Definition
|
|
Term
| 6 entities in the DD of upper lobe pulmonary disease |
|
Definition
| sarcoidosis, hypersensitivity pneumonitis, silicosis, Langerhans cell histiocytosis, tuberculosis and Pneumocystis pneumonia |
|
|
Term
| what is the most sensitive test for an interstitial lung disease such as sarcoidosis |
|
Definition
|
|
Term
| sarcoidosis may be both restrictive and obstructive |
|
Definition
|
|
Term
| what is the name of the specific skin involvement of the nose bridge and area under eyes in sarcoidosis |
|
Definition
|
|
Term
| what does Lofgren's syndrome, a subset of sarcoidosis, consist of |
|
Definition
| hilar adenopathy, erythema nodosum, uveitis |
|
|
Term
| what is the MC chronic skin manifestation in sarcoidosis |
|
Definition
|
|
Term
| what is the MC ocular manifestation in sarcoidosis |
|
Definition
|
|
Term
| what is the MC liver function test affected by sarcoidosis |
|
Definition
|
|
Term
| which is a liver function test that indicates a severe disease in sarcoidosis |
|
Definition
|
|
Term
| what % of sarcoidosis patients have liver involvement if determined by Bx? by liver function tests? |
|
Definition
|
|
Term
| what is the MC hematologic manifestation of sarcoidosis and why? |
|
Definition
| lymphopenia, a reflection of sequestration of the lymphocytes into the areas of inflammation |
|
|
Term
| what % of patients with sarcoidosis have hypercalcemia? what is the mechanism |
|
Definition
| 10%, increased production of 1,25-dihydroxyvitamin D by the granuloma |
|
|
Term
| what is the MC cause of renal failure in sarcoidosis |
|
Definition
|
|
Term
| 4 areas of the CNS that are MC involved in sarcoidosis |
|
Definition
| cranial nerves, basilar meningitis, myelopathy, and anterior hypothalamic disease with associated diabetes insipidus |
|
|
Term
| what is similar btwn neurosarcoidosis and multiple sclerosis |
|
Definition
| MRI findings, optic neuritis |
|
|
Term
| which 2 MRI findings may distinguish sarcoidosis from multiple sclerosis |
|
Definition
| involvement of hypothalamus and meninges |
|
|
Term
| 2 clinical cardiac manifestations of sarcoidosis |
|
Definition
|
|
Term
| sarcoidosis isn't life threatening but it can be organ threatening. which are the 3 most serious complications? |
|
Definition
| blindness, paraplegia, or renal failure |
|
|
Term
| mortality in sarcoidosis is caused by involvement of which 4 organs? |
|
Definition
| lung, cardiac, neurologic, or liver |
|
|
Term
| which lung findings support the Dx of sarcoidosis over other interstitial lung diseases |
|
Definition
|
|
Term
| which 2 imaging modalities are used to assess the spread of sarcoidosis |
|
Definition
|
|
Term
| 5 conditions in which there is more than 50% elevation of the upper limit of normal of ACE |
|
Definition
| sarcoidosis, leprosy, Gaucher's disease, hyperthyroidism, and disseminated granulomatous infections such as miliary tuberculosis |
|
|
Term
| what is the next step in the workup of suspected sarcoidosis in a patient with positive clinical findings and negative pathology/ |
|
Definition
supportive tests: ACE levels > X2 normal
positive gallium scan - increased activity in the parotids and lacrimal glands (panda sign) or in the right paratracheal and left hilar area (lambda sign)
BAL washings showing increased lymphocytes or more specifically a CD4/CD8 ratio > 3.5 |
|
|
Term
| what is the historical diagnostic test, Kveim-Siltzbach, for sarcoidosis |
|
Definition
| spleen tissue from a known sarcoidosis patient is injected intradermally to the suspected patient and Biopsied a month later to reveal non-caseating granulomas |
|
|
Term
| what is the course of disease in most sarcoidosis patients |
|
Definition
| the disease resolves within 2-5 yrs |
|
|
Term
| 6 features that suggest chronic course of disease in sarcoidosis |
|
Definition
| fibrosis on CXR, lupus pernio, bone cysts, cardiac or neurologic disease (except isolated seventh nerve paralysis), and presence of renal calculi due to hypercalciuria |
|
|
Term
| what is the treatment of sarcoidosis with minimal to no manifestations |
|
Definition
|
|
Term
| what is the treatment of sarcoidosis limited to one organ |
|
Definition
|
|
Term
| what is the treatment of choice of sarcoidosis |
|
Definition
|
|
Term
| in what situations should steroids be stopped and alternative drugs initiated in sarcoidosis |
|
Definition
| dose needed over 10mg/d, not effective, not tolerated |
|
|
Term
| 5 steroid sparing agents for the treatment of sarcoidosis |
|
Definition
| MTX, Leflunomide, azathioprine, hydroxychloroquine, minocycline |
|
|
Term
| which anti TNF agent is effective in sarcoidosis and which is not? what other condition shows the same phenomenon? |
|
Definition
infliximab effective, etanercept not
Crohn's disease |
|
|
Term
| the FMF gene encodes a protein called? and situated? |
|
Definition
| pyrin. granulocytes, eosinophils, monocytes, dendritic cells, and synovial and peritoneal fibroblasts |
|
|
Term
| arthritis in FMF is affects which 3 joints |
|
Definition
|
|
Term
| CXR of patients with FMF pleural attack shows which 2 findings |
|
Definition
|
|
Term
| what is the MC skin manifestation in FMF |
|
Definition
| erysipelas-like erythema, most commonly occurs on the dorsum of the foot, ankle, or lower leg |
|
|
Term
| which 2 conditions may be seen at an increased frequency in FMF patients |
|
Definition
| Henoch Schonlein and Polyarteritis nodosa |
|
|
Term
| why does amyloidosis occur in FMF? what organs affected? |
|
Definition
| Amyloid A is an acute phase reactant that is deposited in the kidneys, adrenals, intestine, spleen, lung, and testes |
|
|
Term
| how is the diagnosis of FMF done? |
|
Definition
| clinical findings and genetics |
|
|
Term
| what is the treatment of FMF? |
|
Definition
| colchicin - reduces attack frequency and progression to amyloidosis |
|
|
Term
| colchicine taken by any of the parents during conception for FMF may increase the risk of what condition in the infant |
|
Definition
|
|
Term
| what is the interaction btwn colchicine and cyclosporine |
|
Definition
| cyclosporine inhibits MDR-1 required for hepatic clearance of colchicine causing toxicity |
|
|
Term
| 10 drugs that may cause arthralgias |
|
Definition
| Quinidine, cimetidine, quinolones, acyclovir, interferon, IL-2, nicardipine, vaccines, aromatase and HIV protease inhibitors |
|
|
Term
| 15 drugs that may cause myalgia/myopathy |
|
Definition
| Glucocorticoids, penicillamine, hydroxychloroquine, AZT, statins, clofibrate, interferon, IL-2, alcohol, cocaine, taxol, colchicine, quinolones, cyclosporine, protease inhibitors |
|
|
Term
| 3 drugs that may cause tendon rupture/tenditis |
|
Definition
| Quinolones, glucocorticoids, isotretinoin |
|
|
Term
| 6 drugs that may cause gout |
|
Definition
| Diuretics, aspirin, cytotoxics, cyclosporine, alcohol, ethambutol |
|
|
Term
| 14 drugs that may cause drug-induced lupus |
|
Definition
| Hydralazine, procainamide, quinidine, phenytoin, carbamazepine, methyldopa, isoniazid, chlorpromazine, lithium, penicillamine, tetracyclines, TNF inhibitors, ACE inhibitors, ticlopidine |
|
|
Term
| 4 drugs that may cause osteonecrosis |
|
Definition
| Glucocorticoids, alcohol, radiation, bisphosphonates |
|
|
Term
| 4 drugs that may cause osteopenia |
|
Definition
| Glucocorticoids, chronic heparin, phenytoin, methotrexate |
|
|
Term
| which 3 conditions involve the DIP joint of the hand |
|
Definition
| OA, psoriatic and reactive arthritis |
|
|
Term
| 4 conditions which involve the PIP joint |
|
Definition
| OA, SLE, RA, psoriatic arthritis |
|
|
Term
| 3 conditions which involve the metacarpophalangeal joint |
|
Definition
| RA, pseudogout, hemochromatosis |
|
|
Term
| 5 conditions which involve the wrist |
|
Definition
| carpal tunnel syndrome, RA, gonococcal arthritis, pseudogout, juvenile arthritis |
|
|
Term
| the only condition which involves the 1st carpometacarpal joint of the hand |
|
Definition
|
|
Term
| which imaging modality is diagnostic for tendon injury and rotator cuff tears? |
|
Definition
|
|
Term
| which imaging modality is diagnositic for prosthetic infection and acute osteomyelitis |
|
Definition
| WBC Radionuclide scintigraphy |
|
|
Term
| which imaging modality is diagnostic for paget's disease and chronic osteomyelitis |
|
Definition
| Technecium radionuclide scintigraphy |
|
|
Term
| which imaging modality is diagnostic for avascular necrosis |
|
Definition
|
|
Term
| which 5 joints are MC affected in osteoarthritis |
|
Definition
| cervical and lumbosacral spine, hip, knee, and first metatarsal phalangeal joint (MTP) |
|
|
Term
| symptomatic osteoarthritis of the knee occur in what % of the population |
|
Definition
| 12% under 60 and 6% under 30 |
|
|
Term
| which crystal cannot be detected by polarized light |
|
Definition
|
|
Term
| which joints are commonly involved in gout |
|
Definition
| 1st metatarsalphalangeal joint, tarsal, ankle, interphalangeal in elderly |
|
|
Term
| 6 events may precipitate gout |
|
Definition
| dietary excess, trauma, surgery, excessive ethanol ingestion, hypouricemic therapy, and serious medical illnesses such as myocardial infarction and stroke |
|
|
Term
| the crystals in gout are comprised of what material |
|
Definition
|
|
Term
| what are the typical WBC counts in aspirated joint fluid affected with gout |
|
Definition
|
|
Term
| what are serum uric acid and urate levels in gout |
|
Definition
| uric acid is normal or decreased whereas urate is elevated |
|
|
Term
| what is suggestive of purine overproduction in a person with a normal diet? |
|
Definition
| uricaciduria of more than 800 mg/d |
|
|
Term
| 3 drugs that may be used to treat acute gouty arthritis |
|
Definition
| NSAIDs, colchicine, glucocorticoids |
|
|
Term
| 3 factors that affect the use of prophylactic treatment for gout |
|
Definition
| 2 or over attacks, renal calculi, serum uric acid>9 mg/dl |
|
|
Term
| 2 definite indications for urate-lowering treatment? |
|
Definition
| tophus or chronic gout arthritis |
|
|
Term
| 2 preconditions for the use of probenecid as an urate-lowering treatment |
|
Definition
| uric acid excretion of under 600mg/d and creatinine<2 mg/dl |
|
|
Term
| what are 2 urate-lowering treatment options for patients with renal failure (creatinine>2mg/dl) |
|
Definition
| allopurinol and Benzbromarone |
|
|
Term
| which 3 drugs not routinely used as urate-lowering treatment have such a property? |
|
Definition
| losartan, fenofibrate, and amlodipine |
|
|
Term
| allopurinol may be toxic in what 2 situations |
|
Definition
patients allergic to penicillin and ampicillin
concomitant use of thiazides |
|
|
Term
| 5 serious side effects of allopurinol |
|
Definition
| toxic epidermal necrolysis, systemic vasculitis, bone marrow suppression, granulomatous hepatitis, and renal failure |
|
|
Term
| 7 conditions associated with pseudogout |
|
Definition
aging
Primary hyperparathyroidism
Hemochromatosis
Hypophosphatasia
Hypomagnesemia
Chronic gout
Postmeniscectomy |
|
|
Term
| what material is accumulated in pseudogout |
|
Definition
| calcium pyrophosphate dihydrate CPPD |
|
|
Term
| MC joint involved in pseudogout |
|
Definition
|
|
Term
| what is the definitive diagnosis of pseudogout |
|
Definition
| demonstration of typical rhomboid or rodlike crystals in synovial fluid or articular tissue |
|
|
Term
| what is the treatment of pseudogout |
|
Definition
monoarthritis - NSAIDs
polyarthritis - glucocorticoids, anakinra (il1b antagonist) |
|
|
Term
| 2 known etiologies that cause calcium apatite deposition in joints |
|
Definition
| local tissue damage (dystrophic calcinosis) and hypercalcemic state |
|
|
Term
| what are the synovial fluid leukocyte counts in calcium apatite deposition disease |
|
Definition
|
|
Term
| how is the diagnosis of calcium apatite deposition disease usually done and what are the definitive diagnosis options |
|
Definition
wright stain - purple
electron microscopy, x-ray diffraction, infrared spectroscopy, or Raman microspectroscopy |
|
|
Term
| what condition is directly associated with calcium oxalate deposition disease |
|
Definition
|
|
Term
| calcium oxalate deposits may increase in patients taking what? |
|
Definition
|
|
Term
| what is the leukocyte count in synovial fluid of calcium oxalate affected joint |
|
Definition
| less than 2000 (non-inflammatory) |
|
|
Term
| 3 microscopic features of calcium oxalate deposits |
|
Definition
| bipyramidal, strong birefringence, stain with alizarin red S |
|
|
Term
| typical synovial fluid WBC counts in patients with bacterial infective arthritis |
|
Definition
| around 100,000 and over with >90% neutrophils |
|
|
Term
| typical synovial fluid WBC counts for fungal or mycobacterial infective arthritis |
|
Definition
| WBC 10,000-30,000 with 50-70% neutrophils |
|
|
Term
| 3 MC organisms in hematogenous septic arthritis of the infant |
|
Definition
| group B streptococci, gram-negative enteric bacilli, and S. aureus |
|
|
Term
| 3 MC organisms that cause hematogenous septic arthritis in children under the age of 5 |
|
Definition
| S. aureus, Streptococcus pyogenes (group A Streptococcus), and (in some centers) Kingella kingae |
|
|
Term
| MC organism that causes hemotogenous septic arthritis in young adults |
|
Definition
|
|
Term
| MC organism that causes hematogenous non-gonococcal septic arthritis in adults of all ages |
|
Definition
|
|
Term
| what is the MC route of infection in septic arthritis of all ages |
|
Definition
|
|
Term
| which organisms are MC in septic arthritis following implantation of a prosthetic joint or arthroscopy |
|
Definition
| coagulase negative staphylococci |
|
|
Term
| which organisms are MC in septic arthritis following human bites and abscesses spreading to joints |
|
Definition
| anaerobic and aerobic/facultative flora |
|
|
Term
| specific example of anaerobic organism that may cause septic arthritis following a human bite |
|
Definition
|
|
Term
| which condition is associated with the highest rates of septic arthritis |
|
Definition
|
|
Term
| 5 conditions which carry an increased risk of septic arthritis caused by S.Aureus and gram negative enteric bacilli |
|
Definition
| RA, Diabetes mellitus, glucocorticoid therapy, hemodialysis, and malignancy |
|
|
Term
| 4 conditions which carry increased risk of strep pneumonia septic arthritis |
|
Definition
| HIV, alcoholism, humoral deficiencies, and hemoglobinopathies |
|
|
Term
| HIV patients are at an increased risk of developing septic arthritis d/t 3 organisms |
|
Definition
| s.pneumonia, salmonella, H.influenza |
|
|
Term
| patients with primary immunoglobulin deficiencies carry an increased risk of septic arthritis d/t to which organism |
|
Definition
|
|
Term
| what is the treatment of mycoplasma septic arthritis in the patient with a primary immunoglobulin deficiency |
|
Definition
|
|
Term
| 3 joints that are MC affected in IV drug users with septic arthritis |
|
Definition
| spine, sacroiliac joints, and sternoclavicular joints |
|
|
Term
| what underlying condition carries the highest risk of polyarticular septic arthritis |
|
Definition
|
|
Term
| 3 conditions in which concomitant septic arthritis may present w/o fever |
|
Definition
| rheumatoid arthritis, renal or hepatic insufficiency, or conditions requiring immunosuppressive therapy |
|
|
Term
| 3 radiographic findings in septic arthritis |
|
Definition
| soft tissue swelling, joint-space widening, and displacement of tissue planes by the distended capsule |
|
|
Term
| 3 findings in chemistry of synovial fluid aspirated in septic arthritis |
|
Definition
| elevated protein and LDH and decreased glucose |
|
|
Term
| in septic arthritis, what is an adequate empirical coverage for most community-acquired infections in adults when smears show no organisms |
|
Definition
| 3rd generation cephalosporins: cefotaxime/ceftriaxone |
|
|
Term
| in septic arthritis, what is an adequate empirical coverage for most community-acquired infections in adults when smears show gram-positive cocci and the community is contaminated with MRSA |
|
Definition
|
|
Term
| in septic arthritis, what is an adequate empirical coverage for most community-acquired infections in adults when smears show gram-positive cocci if MRSA is an unlikely pathogen |
|
Definition
|
|
Term
| in septic arthritis, what is an adequate empirical coverage for most community-acquired infections in adults when pseudomonas is possible such as in IV drug users |
|
Definition
| aminoglycoside and Ceftriaxone |
|
|
Term
| when are women at the greatest risk to develop disseminated gonococcal infection syndrome |
|
Definition
|
|
Term
| which immunodeficiency imposes to gonococcal septic arthritis |
|
Definition
| complement deficiencies - terminal components |
|
|
Term
| the rash in disseminated gonococcal infection syndrome tends to involve which areas |
|
Definition
| trunk and the extensor surfaces of the distal extremities |
|
|
Term
| what is the pathophysiology of disseminated gonococcal infection syndrome (vs. true gonococcal septic arthritis) |
|
Definition
| immune reaction to deposition of immune complexes in joints |
|
|
Term
| what do cultures of disseminated gonococcal infection syndrome show (vs. true gonococcal septic arthritis)? |
|
Definition
| nothing. they're negative |
|
|
Term
| what are the typical WBC counts in the synovial fluid of disseminated gonococcal infection syndrome (vs. true gonococcal septic arthritis) |
|
Definition
|
|
Term
| 2 articular manifestations of disseminated gonococcal infection syndrome (vs. true gonococcal septic arthritis) |
|
Definition
| Migratory arthritis and tenosynovitis |
|
|
Term
| true gonococcal septic arthritis always follows... |
|
Definition
| disseminated gonococcal infection |
|
|
Term
| 4 MC joints involved in true gonococcal septic arthritis |
|
Definition
|
|
Term
| WBC counts in synovial fluid aspirate from true gonococcal septic arthritis |
|
Definition
|
|
Term
| blood cultures in true gonococcal septic arthritis |
|
Definition
|
|
Term
| synovial joint fluid cultures in true gonococcal septic arthritis are positive in... |
|
Definition
|
|
Term
| since blood and synovial cultures are frequently negative in true gonococcal septic arthritis what is a diagnostic step |
|
Definition
|
|
Term
| how is culture preformed in suspected gonococcal infection |
|
Definition
| martin-theyer agar, 5% CO2 |
|
|
Term
| Tx of gonococcal proven septic arthritis |
|
Definition
| IV or IM Ceftriaxone until remission of symptoms and then a course of 7 days ciprofloxacin and doxycycline (the latter for chlamydia) |
|
|
Term
| what is the course of untreated lyme disease (borrelia burgdorfferi) in terms of joint involvement |
|
Definition
| first 3-4 wks of inoculation - arthralgia later on may develop into monoarthritis, synovitis which will destruct the joint or wax and wane arthralgia |
|
|
Term
| what test is positive for borrelia burgdorfferi in 90% of cases |
|
Definition
|
|
Term
| 3 treatment options for lyme arthritis |
|
Definition
| IV Ceftriaxone, oral doxycycline, oral amoxicillin |
|
|
Term
| what are 2 treatment options of lyme arthritis in case of failure with oral Abx therapy after 2 months or parenteral after one month? |
|
Definition
| anti inflammatory agents or synovectomy |
|
|
Term
| 2 features of joint involvement in secondary syphillis |
|
Definition
|
|
Term
| 2 characteristics of arthritis in secondary syphillis |
|
Definition
| bilateral and subacute/chronic |
|
|
Term
| what are the cell counts present in syphillitic arthritis |
|
Definition
| 10,000 WBC mixed monocytes and neutrophils |
|
|
Term
| what is the treatment of arthritis caused by secondary syphillis |
|
Definition
|
|
Term
| what is a joint involvement of tertiary syphillis |
|
Definition
| charcot's joints - unresponsive to penicillin |
|
|
Term
| 2 joint manifestations in congenital syphillis |
|
Definition
1. early - periarticular swelling and immobilization of the involved limbs (Parrot's pseudoparalysis)
2. ages 8-15 - Clutton's joint, chronic painless synovitis with effusions of large joints |
|
|
Term
| what is the MC presentation of tuberculous arthritis |
|
Definition
|
|
Term
| what is poncet's disease in visceral or disseminated TB? |
|
Definition
| reactive symmetric form of polyarthritis |
|
|
Term
| which 3 joints are primarily involved in tuberculous arthritis? |
|
Definition
| weight bearing joints: ankle, knee, hip |
|
|
Term
| what % of patients with tuberculous arthritis experience systemic symptoms |
|
Definition
|
|
Term
| what is the cell count in tuberculous arthritis |
|
Definition
|
|
Term
| which method can shorten time of diagnosis of tuberculous arthritis |
|
Definition
|
|
Term
| what type of joint involvement can fungus cause |
|
Definition
|
|
Term
| 4 fungus species that may cause chronic monoarthritis |
|
Definition
| Coccidioides immitis, Blastomyces dermatitidis, Histoplasma capsulatum, Sporthrix Schenckii |
|
|
Term
| what is the treatment of fungal arthritis |
|
Definition
| IV + intraarticular Amphotericin B |
|
|
Term
| 4 MC enteric bacteria that elicit reactive arthritis |
|
Definition
| Yersinia enterocolitica, Shigella flexneri, Campylobacter jejuni, and Salmonella |
|
|
Term
| what is the MC complaint in fibromyalgia |
|
Definition
|
|
Term
| what is the pharmacologic approach to treatment of fibromyalgia |
|
Definition
| SNRIs (amitryptiline) or anticonvulsants (gabapentin) |
|
|
Term
| 2 joint manifestations in acromegaly |
|
Definition
| osteoarthritis and back pain d/t hypermobility of spine (widened articular spaces d/t more cartilage) |
|
|
Term
| 4 musculoskeletal problems in acromegaly |
|
Definition
| osteoarthritis, back pain, muscle weakness, and carpal tunnel syndrome |
|
|
Term
| what is the dynamics of arthropathy in hemochromatosis |
|
Definition
| osteoarthritis-like starting in small joints (hands) and progressing to larger ones |
|
|
Term
| which joints are primarily affected in hemochromatosis associated arthropathy |
|
Definition
| 2nd and 3rd metacarpophalangeal |
|
|
Term
| what are the radiographic findings in hemochromatosis associated arthropathy |
|
Definition
|
|
Term
| what is the position of the joint held by the patient affected by hemarthrosis? |
|
Definition
|
|
Term
| how is bleeding into the iliopsoas distinguished from hip hemarthrosis or hip synovitis of any cause? |
|
Definition
| hip is kept in flexion in all cases. in bleeding into iliopsoas hip rotation is preserved |
|
|
Term
| what is a painful pseudotumor distal to the elbows or knees in children |
|
Definition
| bleeding into the periosteum in a child with hemophilia |
|
|
Term
| what is the pharmacological approach to hemearthrosis |
|
Definition
1st give clotting factors
2nd give selective COX2 inhibitors - do not inhibit platelets |
|
|
Term
| if the pharmacological approach fails in hemarthrosis, what is the next approach |
|
Definition
|
|
Term
| which musculoskeletal manifestation is observed in children up to the age of five with sickle cell disease |
|
Definition
| sickle cell dactylitis - metacarpals, metatarsals, and proximal phalanges - infarction of the bone marrow leading to periostitis and soft tissue swelling |
|
|
Term
| 2 radiographic findings in sickle cell dactylitis |
|
Definition
| subperiosteal new bone formation and areas of radiolucency and increased density |
|
|
Term
| what is the natural history of sickle cell dactylitis |
|
Definition
| goes away after a few months w/o leaving a mark |
|
|
Term
| 2 features of joint involvement in sickle cell crisis. what joints are MC involved/ |
|
Definition
| periarticular pain and joint effusion. usually knees or elbows |
|
|
Term
| one radiographic similarity and one distinguishing feature of osteomyelitis vs. bone infarction in sickle cell disease |
|
Definition
similar - periosteal elevation
different - in bone infarction: new cortical bone formation. in osteomyelitis - disruptions of cortical bone |
|
|
Term
| MC site of avascular necrosis in sickle cell disease patients. prevalence? |
|
Definition
|
|
Term
| 3 organisms which are MC responsible for septic arthritis in sickle cell disease |
|
Definition
| Staphylococcus aureus, Streptococcus, and Salmonella |
|
|
Term
| what is more common salmonella septic arthritis or osteomyelitis in sickle cell disease? |
|
Definition
|
|
Term
| 2 secondary bone changes d/t marrow hyperplasia in sickle cell disease |
|
Definition
| vertebral compression leading to kyphosis and acetabulum softening leading to protrusio acetabuli |
|
|
Term
| musculoskeletal manifestations in beta Thalassemia |
|
Definition
1. symmetric ankle arthropathy d/t bone marrow expansion (minifractures)
2. secondary hemochromatosis and hemarthrosis |
|
|
Term
| joint involvement in hypercholesterolemia |
|
Definition
| migratory polyarthritis of kness and other large joints |
|
|
Term
| what are the physical findings in migratory polyarthritis d/t familial hypercholesterolemia |
|
Definition
| warm, erythematous, swollen, and tender |
|
|
Term
| natural history of arthritis d/t familial hypercholesterolemia |
|
Definition
| abrupt onset, lasts days to wks |
|
|
Term
| when do xanthomas appear on the tendons in familial hypercholesterolemia? |
|
Definition
| in homozygotes - childhood, in heterozygotes - after the age of 30 |
|
|
Term
| defintion of charcot's joint/neuropathic joint |
|
Definition
| progressive destructive arthritis associated with loss of pain sensation, proprioception, or both |
|
|
Term
| 9 conditions that may manifest charcot's joint |
|
Definition
| DM, Tabes Dorsalis, leprosy, yaws, syringomyelia, meningomyelocele, congenital indifference to pain, peroneal muscular atrophy (Charcot-Marie-Tooth disease), and amyloidosis |
|
|
Term
| MC condition that may manifest charcot's joint |
|
Definition
|
|
Term
| which joint are MC involved in diabetes associated charcot's joint |
|
Definition
| tarsal and tarsometatarsal joints |
|
|
Term
| which joints are MC involved in Tabes Dorsalis associated charcot's joint |
|
Definition
|
|
Term
| physical finding on foot of diabetic patient with charcot's joint |
|
Definition
| convexity of the sole - "rocker foot" |
|
|
Term
| what is Lisfranc fracture-dilocation |
|
Definition
| destructive changes at the tarsometatarsal joints in DM associated charcot's joint |
|
|
Term
| how to distinguish radiographically btwn osteomyelitis and neuropathic joint in the foot of a diabetic |
|
Definition
| The joint margins in a neuropathic joint tend to be distinct, while in osteomyelitis, they are blurred |
|
|
Term
| 3 ways to distinguish osteomyelitis from neuropathic joint in the foot of a diabetic |
|
Definition
1. radiographic imaging
2. bone scan
3. joint aspiration |
|
|
Term
| what is the treatment of a patient with diabetes associated neuropathic joint |
|
Definition
| immobilization of the joint |
|
|
Term
| what is pachydermoperiostitis or Touraine-Solente-Golé syndrome |
|
Definition
| a congenital form of hypertrophic osteoarthropathy (includes clubbing) |
|
|
Term
| 2 physical examination tests for clubbing |
|
Definition
| nail easily rocked on distal phalanx and diameter at base of nail greater than DIP joint |
|
|
Term
| what are the 2 most common intrathoracic tumors causing hypertrophic osteoarthropathy (includes clubbing) |
|
Definition
| bronchogenic carcinoma and pleural tumors |
|
|
Term
| which pulmonary conditions do not cause clubbing |
|
Definition
|
|
Term
| which 3 types of GI conditions cause clubbing |
|
Definition
| IBD, celiac, malignancy (esophagus, liver, bowel) |
|
|
Term
| 6 cardiovascular conditions that can cause clubbing |
|
Definition
cyanotic congenital heart disease
aneurysms
stent infection
A-V fistula
patent ductus arteriosus
subacute bacterial endocarditis |
|
|
Term
| which endocrinologic disease may cause clubbing? what is it called? |
|
Definition
| Grave's disease, "thyroid acropachy" |
|
|
Term
| what is the radiographic finding in hypertrophic osteoarthropathy |
|
Definition
| a faint radiolucent line beneath the new periosteal bone along the shaft of long bones at their distal end (ankles, knees, wrists) |
|
|
Term
| what is Reflex Sympathetic Dystrophy Syndrome |
|
Definition
| nerve damage at distal extremities causes pain and development of bony demineralization |
|
|
Term
| what is the MC form of bursitis |
|
Definition
|
|
Term
| which maneuvers elicit pain in trochanteric bursitis |
|
Definition
| external rotation and abduction against resistance |
|
|
Term
| which muscle is MC involved in impingement syndrome of the shoulder |
|
Definition
|
|
Term
| what is a challenge test for supraspinatus impingement syndrome |
|
Definition
| forward elevation of the arm |
|
|
Term
| which muscle is MC involved in calcific tenditis caused by ischemic injury |
|
Definition
|
|
Term
| a test to determine bicipital tendinitis |
|
Definition
| resisting supination of the forearm with the elbow at 90° (Yergason's supination sign) |
|
|
Term
| what is the finkelstein test to determine De Quervian tenosynovitis |
|
Definition
| place thumb in palm and squeeze over with fingers |
|
|
Term
| what is iliotibial band syndrome |
|
Definition
| the band spans from the ileum to the fibula. pain is felt where it crosses over the femoral lateral condyle and radiates up to the side of the thigh |
|
|
Term
| what is the treatment of choice of wegener's granulomatosis |
|
Definition
|
|
Term
| IM ACTH may be used in acute gout |
|
Definition
|
|
Term
| secondary prevention of acute rheumatic fever/rheumatic heart disease |
|
Definition
|
|
Term
| what is the initial treatment of patients with pulmonary artery hypertension and underlying systemic sclerosis |
|
Definition
| endothelin-1 receptor antagonist or a phosphodiesterase inhibitor such as sildenafil |
|
|
Term
| what is the treatment of ankylosing spondylitis that is unresponsive to NSAIDs? |
|
Definition
|
|
Term
| treatment of whipples disease |
|
Definition
| penicillin (or ceftriaxone) and streptomycin for 2 weeks followed by trimethoprim-sulfamethoxazole for 1–2 years |
|
|
Term
| initial treatment of choice for inflammatory myopathies: DM, PM, IBM |
|
Definition
|
|
Term
| 4 step treatment model for inflammatory myopathies and when complicated by interstitial lung disease |
|
Definition
| Step 1: high-dose prednisone; Step 2: azathioprine, mycophenolate, or methotrexate for steroid-sparing effect; Step 3: IVIg; Step 4: a trial, with guarded optimism, of one of the following agents, chosen according to the patient's age, degree of disability, tolerance, experience with the drug, and general health: rituximab, cyclosporine, cyclophosphamide, or tacrolimus. Patients with interstitial lung disease may benefit from aggressive treatment with cyclophosphamide or tacrolimus |
|
|
Term
| what is the most sensitive lab test for SLE |
|
Definition
|
|
Term
| what is the treatment of polymyalgia rheumatica |
|
Definition
|
|
Term
| which serologic marker in RA predicts poorer prognosis and erosions? |
|
Definition
|
|
Term
| which type of crystal deposition disease is associated with osteoarthritis |
|
Definition
|
|
Term
| what are 3 indications for the use of allopurinol+azathioprine and what is a side effect |
|
Definition
IBD, prevention of rejection in organ transplantation, and reducing thiopurine-induced hepatotoxicity
Granulocytopenia |
|
|
Term
| what malignancy is associated with RA |
|
Definition
|
|
Term
| injury to heart in reactive arthritis |
|
Definition
|
|
Term
| FMF can manifest with a painful rash on the lower extremities |
|
Definition
|
|
Term
| what is the mechanism of anemia MC in SLE |
|
Definition
| of chronic disease (not hemolytic) |
|
|
Term
| erosions are rare in SLE induced arthropathy |
|
Definition
|
|
Term
| when do you opt for second line treatment in sarcoidosis? what is the first line? |
|
Definition
first line - steroids
second line - when cannot taper steroids to under 10 mgs: MTX, azathioprine, leflunomide, hydroxychloroquine, minocycline |
|
|
Term
| what are 4 3rd line treatment options for sarcoidosis |
|
Definition
| multiple 2nd line agents, thalidomide, infliximab, cyclophosphamide |
|
|
Term
| neurologic findings in behchet |
|
Definition
| sinus thrombosis, brain stem, white matter involvement |
|
|
Term
| how do you treat oral mucous membrane involvement in behcet? |
|
Definition
|
|
Term
| how do you treat thrombophlebitis in behcet |
|
Definition
|
|
Term
| how do you treat uveitis and CNS manifestations in behcet |
|
Definition
|
|
Term
| how do you treat pulmonary or peripheral arterial aneurysms in behcet |
|
Definition
| pulse doses of cyclophosphamide |
|
|
Term
| what do you do before initiating treatment for lupus nephritis |
|
Definition
| renal biopsy to determine if it is class III or IV that will require added cyclophosphamide |
|
|
Term
|
Definition
| exudative, monocytosis, neutrophils |
|
|
Term
| CXR and LFT abnormalities are not indication enough for treatment in sarcoidosis |
|
Definition
|
|
Term
|
Definition
|
|
Term
| the lumbar spine is not affected in RA |
|
Definition
|
|
Term
| NSAIDs do not affect the natural course of RA |
|
Definition
|
|
Term
| how does synovial fluid in RA differ from OA? |
|
Definition
| 5,000-50,000 WBC in RA vs. >2000 in OA |
|
|
Term
| diagnosis of Takayasu arteritis - 3 out of 6 criteria |
|
Definition
o נשים תחת גיל 40
o הפרעה בתנועת גפיים בכלל כמו בידיים או צליעה לסירוגין
o הפרעה בדופק ברכיאלי או העדרו (pulseless disease)
o שינויים בל"ד בין שתי הידיים הגדולים מ- 10 מ"מ כספית
o אוושה בעורק ה-subclavian
o ארטריוגרפיה אופיינית - היצרות האאורטה או סעיפיה |
|
|
Term
|
Definition
• אוסטאופניה juxtaarticular
• אובדן סחוס במפרק וארוזיה של עצם |
|
|
Term
|
Definition
| מדכא סימנים וסימפטומים של דלקת ומעכב ארוזיות בעצם ומעלה אפקטים אנטי דלקתיים של MTX |
|
|
Term
|
Definition
| NSAIDs, סטרואידים, DMARDS, ביולוגי, אימונוסופרסיבי (LEFLUNOMIDE - pyrimidine synthesis inhibitor) |
|
|
Term
|
Definition
• קליני (צריך 1): אירוע טרומבוטי ורידי או עורקי או תחלואה מיילדותית מוגדרת היטב
מות עובר בשבוע 10 ומעלה - רוב ההפלות באוכ' שנובעות מבעיות כרומוזומליות מתרחשות עד שבוע 10 ולכן אם ההפלה ארעה אחרי שבוע 10 צריך לחפש APLA
רעלת הריון מתחת לשבוע 34 – בד"כ קורה אחרי שבוע 34, אם קורה לפני לבדוק APLA.
3 הפלות חוזרות מתחת לשבוע 10
אי ספיקה של השיליה לפני שבוע 34.
בכל מקרה צריך לשלול סיבה אחרת לבעיות בהיריון.
אם זו טרומבוזה צריך לשלול סיבה אחרת - גנטי בצעירים ובמבוגרים מחפשים יתר לחץ דם, מחפשים גורם אחר לאירוע טרומבוטי - בנשים מעל גיל 65 וגברים 55.
• מעבדתי (צריך 1): Anti cardiolipin IgG, Anti cardiolipin IgM, Lupus anti coagulant, Anti B2 GP1- הנוגדנים מאוד הטרוגנים ושונה מחולה לחולה. צריך תוצאה חיובית לפחות פעמיים בהפרש של 12 שבועות, וטיטר גבוה מעל 40. יכול להיות חולף בגלל דלקת או תרופות. |
|
|
Term
| renal manifestations in PAN |
|
Definition
| o בכליה יש ארטריטיס לא GN. הביטוי הכלייתי הוא בעיקר יל"ד, אי ספיקת כליות או דימום |
|
|
Term
|
Definition
|
|
Term
|
Definition
| כיבי גניטליה פחות נפוצים אך יותר ספציפים, לא פוגעים ביורטרה וגלנס פניס |
|
|
Term
| מניפסטציות עיניות בסרקואידוזיס |
|
Definition
o אובאיטיס קדמי-הכי שכיח
o אובאיטיס אחורי –ב25 אחוז
o פוטופוביה
o דמעת
o ראיה מטושטשת
o יכול להתקדם לעיוורון
o עניים יבשות בחצי מהחולים הכרונים |
|
|
Term
| 3 סממנים של תסמונת לופגרן כחלק מסרקואידוזיס |
|
Definition
o אריתמה נודוזום
o אובאיטיס
o אדנופתיה הילארית |
|
|
Term
|
Definition
• שחפת
• סטרפטוקוק
• IBD
• סרקואידוזיס
• צרעת
• מיקופלזמה פנאומוניה
• גלולות
• הריון
• בכצט |
|
|
Term
|
Definition
• זיהום קשה כמו לגיונלה או ליסטריה, כולל רהאקטיבציה של שחפת
• מעלה סיכוי ללימפומות או ממאירות אחרת
• לופוס תרופתי-מפתח נוגדנים כנגד anti dna
• מחלת דה מיאלינציה בCNS
• החמרה של אי ספיקת לב
• מחלת כבד קשה
• בעיות המטולוגיה כולל פאנציטופניה |
|
|
Term
| תופעות לוואי של סטרואידים בעובר |
|
Definition
| משקל לידה נמוך, אבנורמליות בהתפתחות CNS, נטיה לסינדרום מטבולי במבוגר |
|
|
Term
| טיפול מועדף ללופוס בהריון |
|
Definition
|
|
Term
| מבחן לקביעה האם תופעת רנו היא ראשונית או שניונית (לסקלרודרמה בד"כ) |
|
Definition
| מיקרוסקופיה של הציפורן. אם קפילרות נורמליות אז מחלה ראשונית. בסקלרודרמה ומחלות רקמת חיבור הקפילרות הרוסות עם loopsאי רגולרים,לומן מורחב ואזורים נטולי קפילרות |
|
|
Term
| תופעת רנו חמורה יותר בתת סוג המוגבל של סקלרודרמה |
|
Definition
|
|
Term
|
Definition
• נוגדן כנגד אנטיגן עצמי
• הנוכחות של ANTI CCP היא הכי נפוצה בחולים עם מחלה RAאגרסיבית עם נטייה לפתח ארוזיות גרמיות-במיוחד בטיטרים גבוהים
• הנוכחות של נוגדן זה נראית באופן הנפוץ ביותר בחולי RA עם HLA B1 ובאלו שמעשנים
• מופיע ב1.5 אחוז מאוכלוסיה בריאה שרובם לא יפתחו RA או מופיע במחלות ראמטולוגיות אחרות
• לרוב מופיע כשיש RF, אך יכול להיות בהיעדרו
• רגישות דומה לRF אך ספציפיות טובה יותר, עוזר לאבחן RA מוקדם |
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|
