Term
| What is characterized by reduced lung volumes b/c of an alteration in lung parenchyma or b/c of a disease of the pleura, chest wall, or neuromuscular apparatus? |
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Definition
| restrictive pulmonary disease |
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Term
| Reduced total lung capacity is characteristic of what type of pulmonary disease? |
|
Definition
| restrictive pulmonary disease |
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Term
| Preserved airway resistance (functional residual capacity (frc)) is characteristic of what type of pulmonary disease? |
|
Definition
| restrictive pulmonary disease |
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Term
| Reduced gas transfer that significantly worsens w/exertion is characteristic of what type of pulmonary disease? |
|
Definition
| restrictive pulmonary disease |
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Term
| What is characterized by loss of lung compliance? |
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Definition
| restrictive pulmonary disease |
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Term
| What are a few collagen vascular disorders that can cause intrinsic lung disease? |
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Definition
| scleroderma, sle, ra, ankylosing spondylitis |
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Term
| What are some medications that cause intrinsic lung disease? |
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Definition
| *amiodarone*, phenytoin, methotrexate |
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Term
| What are some primary diseases that cause intrinsic lung disease? |
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Definition
| sarcoidosis, vasculitis, alveolar proteinosis |
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Term
| What inorganic dust exposures can cause intrinsic lung dissease? |
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Definition
|
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Term
| What organic dust exposure can cause intrinsic lung disease? |
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Definition
| farmer's lung, bagassosis, mushroom worker's lung, hsn pneumonia |
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Term
| What are 3 idiopathic fibrotic disorders that can cause intrinsic lung disease? |
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Definition
| fibrosing interstitial pneumonia, interstitial pneumonitis, lymphocytic interstitial pneumonitis |
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Term
| What are 4 extrinsic disorders that can result in restrictive pulmonary disease? |
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Definition
| atelectasis, kyphoscoliosis, sleep disorders, neuromuscular disease |
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Term
| Extrinsic disorders that result in lung restriction can also cause what? |
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Definition
| impaired ventilatory function, and respiratory failure |
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Term
| What are some important factors to pay careful attention to when taking a hx of a pt w/suspected restrictive pulmonary disease? |
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Definition
| occupation, travel, smoking, hobbies, exposures, hiv risk factors, medication use |
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Term
| A pt presents w/progressive exertional dyspnea, dry cough, hemoptysis, wheezing (rare) and (pleuritic) chest pain (rare). What leads your d/dx? |
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Definition
| intrinsic disease (causing restrictive lung disease) |
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Term
| Pt presents w/kyphoscoliosis, spinal deformity, mm weakness, and a neuromuscular disorder, causing doe, dyspnea at rest and respiratory failure (very sick pt.) Of what disease might these be s/x? |
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Definition
| extrinsic disease (leading to restrictive pulmonary disease) |
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Term
| Massively obese pt w/chest wall abnormality presents w/rhonchi, digital clubbing, cyanosis at rest, and the following extrapulmonary findings: erythema nodosum (sarcoidosis), maculopapular rash (connective tissue disease) and cor pulmonale. What illness might you suspect? |
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Definition
| intrinsic disorder (causing restrictive pulmonary disease) |
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Term
| You perform a pe and find severe kyphoscoliosis, massive obesity,, decreased tactile fremitus, dull percussion, decreased breath sounds, and neuromuscular disease (shown by accessory mm use, rapid shallow breathing and paradoxical respirations). What illness might you suspect? |
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Definition
| extrinsic disorders (causing restrictive pulmonary disease) |
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Term
| You order labs that show anemia (vasculitis), polycthemia (hypoxemia), leukocytosis (pneumonitis). What type of pulmonary disease might you suspect? What additional studies might you order? |
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Definition
| intrinsic disorders (causing restrictive pulmonary disease). Also order antinuclear Abs and Rh factor (collagen vascular disorders), creatine kinase (polymyositis), antineutrophilic cytoplasmic Ab's (vasculitis) and antiglomerular membrane Ab (goodpasture syndrome) |
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Term
| What are 2 imaging studies that might be used to evaluate intrinsic disorders that cause restrictive pulmonary disease? What else might you order? |
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Definition
| cxr, ct (sometimes helpful in dx disease). also lung bx. |
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Term
| What are 4 tx for intrinsic disorders (causing restrictive lung disease)? |
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Definition
| avoid or eliminate the harmful exposure, protective gear (masks, respiators), oral steroids (prednisone), and oxygen |
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Term
| What is referred to as coal miners lung, black lung, silicosis, and asbestosis? |
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Definition
|
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Term
| What involves inhaled particles that set into the lung tissue, causing scarring and fibrosis to take over? |
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Definition
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Term
| Pt presents w/ the following s/s: cough (dry or productive), dyspnea, doe, and fatigue. Pt has worked in a shipyard. What general disorder might this pt have? What aspect of your exam is particularly important? What tests might you order? What are 4 tx you might recommend? |
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Definition
| pneumoconiosis. careful hx taking in pts (occupation and risk exposure). cxr, lung bx. tx: symptomatic, ox tx if needed, smoking cessation, reduce the risk of exposure. |
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Term
| What is a systemic disease involving granulomatous inflammation of the lung? |
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Definition
|
|
Term
| what can be the etiology of sarcoidosis? |
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Definition
| can be genetic etiology- esp in eye and liver involvement |
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|
Term
| What parts of the body are involved in sarcoidosis? |
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Definition
| skin. eyes. peripheral nn. liver. kidney. heart. |
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Term
| Black female 38y pt presents w/fever, malaise, dyspnea, erythema nodosum, parotid gland enlargement, hepatosplenomegaly. What disease might you suspect? What lab abnormalities might you suspect? |
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Definition
| sarcoidosis (other organ systems may present prior to pulmonary complaint). Lab testing: leukocytosis, esr, hypercalcemia (5%), hypercalciuria (20%), elevated antiotensin converting enzyme level (40-80% of population) |
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Term
| What can cxr reveal in stage I sarcoidosis? |
|
Definition
| bilateral hilar adenopathy |
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|
Term
| What can cxr reveal in stage II sarcoidosis? |
|
Definition
| hilar adenopathy or parenchymal involvement |
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|
Term
| What can cxr reveal in stage III sarcoidosis? |
|
Definition
| parenchymal involvement alone |
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|
Term
| What procedure might help you dx sarcoidosis and rule out other pathologies? |
|
Definition
|
|
Term
| What are some tx options for sarcoidosis? |
|
Definition
| oral steroids (prednisone), disabling constitutional sx, tx other parallel problems related to disease (ca2+, heart, cns optho), follow ace levels. |
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Term
| What are 3 types of pulmonary vasulitis? |
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Definition
| 1. wegener's granulmatosis. 2. idiopathic. 3. allergic angiitis and granulmotosis (churg-strauss syndrome) |
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Term
| Pt presents w/ chronic sinusitis, arthralgias, fever, weight loss, and skin rashes. You discover necrotizing granulomatous vasculitis of upper and lower respiratory tract. What illness might you suspect? |
|
Definition
| idipathic pulmonary vasculitis |
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Term
| When might you ssee honeycombing type of lung involvement- fibrotic tissue clustering? |
|
Definition
| sarcoidosis (stage III on cxr) |
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Term
| What is the classic triad of pathologic features for wegener's granulomatosis? |
|
Definition
| 1. systemic necrotizing angiitis. 2. necrotizing granulomatous inflammation of the respiratory tract *upper and lower*. 3. necrotizing glomerulonephritis. |
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Term
| What are the 3 phases of tx for wegener's granulomatosis? |
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Definition
| 1. induction of remission. 2. maintenance of remission. 3. tx of relapse |
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|
Term
| Does wegener's present in the upper or lower respiratory tract? |
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Definition
|
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Term
| How might you tx pulmonary vaculitis? |
|
Definition
| oral steroids (prednisone) for a duration of 3-6 mos. in several cases, iv methylprednisolone (solumedrol) up to 1 gram/day for several days |
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Term
| What is the goal of tx w/fixed lung disease? |
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Definition
| you want to keep disease from progressing or perhaps induce remission. *you aren't trying to cure this.* (peak flow doesn't help at all in wegeners). |
|
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Term
| What is the most common presentation of pts w/interstitial lung disease? |
|
Definition
| idiopathic pulmonary fibrosis |
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|
Term
| In idiopathic pulmonary fibrosis, how are 90% of dx made? How is dx confirmed? |
|
Definition
| important to take a careful hx in determining dx: 90% dx from hx. lung bx helps confirm dx |
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Term
| What are tx options for idiopathic pulmonary fibrosis? |
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Definition
| controversial: oral steroids upon dx for the fist 2 mos (prednisone 1-2 mg/kg/day). ongoing research w/abx. o2 tx for sx relief. in severe cases and no contraindications, lung transplant. |
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Term
| What illness involves blunted drive along w/increased mechanical load on chest wall with voluntary hyperventilation to compensate back into nml po2 and pco2 levels? What do most of these pts also have? |
|
Definition
| obesity-hypoventilation syndrome (pickwickian). these pts normally also have osa. |
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Term
| What do most pts w/obesity-hypoventilation syndrome (pickwickian) also have? |
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Definition
|
|
Term
| What is the goal of obesity-hypoventilation syndrome (pickwickian) tx? |
|
Definition
| weight loss, reduction of obstructive components |
|
|
Term
| What are 4 possible causes of hyperventilation syndrome? |
|
Definition
| pregnancy, sepsis, hypoxemia, obstructive lung disease |
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Term
| In hyperventilation syndrome, increase in ventilation leads to what? |
|
Definition
|
|
Term
| What are tx options for hyperventilation syndrome? |
|
Definition
| in some cases, tx underlying cause. for non-organic disease, anxiolytics and/or brown paper bag to decrease resp. alkalosis |
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|
Term
| What are other factors causing osa? |
|
Definition
| micrognathia, macroglossia, obesity, tonsillar hypertrophy. other factors to consider, hypothyroidism, smoking hx, ?seizure disorder, narcolepsy, depression |
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|
Term
| What involves upper airway obstruction during sleep? |
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Definition
|
|
Term
| The following are factors in what illness: micrognathia, macroglossia, obesity, tonsillar hypertrophy. other factors to consider, hypothyroidism, smoking hx, ?seizure disorder, narcolepsy, depression? |
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Definition
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|
Term
| Pt presents w/ systemic htn, daytime somnolence, fatigue, cognitive impairment, recent weight gain, and snoring. PE reveals obesity, sleepy appearance, narrowed oropharynx, lg soft tissue folds, and "bull neck". What leads your d/dx? |
|
Definition
|
|
Term
| Might a pe for osa be normal? |
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Definition
|
|
Term
| What lab testing modality might be ordered for osa? |
|
Definition
| polysomnography (sleep study) |
|
|
Term
| what are some tx options for osa? |
|
Definition
| tx underlying cause (weight reduction #1), continuous positive airway pressure (cpap) (issue- compliance), uvulopalatopharygeoplasty (uppp), nasal septoplasty, tracheotomy (severe cases) |
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|
Term
| What type of restrictive pulmonary disorder is indicated by antiglomerular membrane Ab? |
|
Definition
|
|
Term
| What type of restrictive pulmonary disorder is indicated by antineutrophilic cytoplasmic Ab's? |
|
Definition
|
|
Term
| What type of restrictive pulmonary disorder is indicated by creatine kinase? |
|
Definition
|
|
Term
| What type of restrictive pulmonary disorder is indicated by Abs and Rh factor? |
|
Definition
| (collagen vascular disorders) |
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