Term
| Name 6 autoimmune diseases |
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Definition
1. Diabetes
2. Systemic Lupus
3. Multiple Sclerosis
4. Rheumatoid Arthritis
5. Graves Disease
6. Myasthenia Gravis |
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Term
| What are autoimmune diseases characterized by? |
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Definition
| non-protective immunopathology |
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Term
| What are the two theories of development of autoimmune diseases? |
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Definition
1. The release of sequestered antigens
2. Molecular mimicry |
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Term
Development of Autoimmune Disease:
1. Release of sequestered antigens |
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Definition
- Response to antigens that aren't normally seen by the immune system are released
- (if an antigen is produced in a particular area of the body and doesn't go into circulation, the immune system doesn't have to learn to respond to it) |
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Term
| If you have a ___ amount of antigen (that is not normally seen) released it's not a problem, but if you have a ___ released this causes disease. |
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Definition
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Term
| After T cells have matured in the thymus, what 2 characteristics are important to have? |
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Definition
- want them to respond to MHC
- don't want to respond to self antigens (no negative selection) |
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Term
What is negative selection?
Where do we see this? |
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Definition
t cells responding to self antigens
In the thymus |
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Term
| What is an example of something that could cause a release of antigen? |
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Definition
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Term
| If you release antigen that hasn't been seen before, in a reasonable amount, in a site of inflammation what will happen? |
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Definition
| get danger signals and an immune response |
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Term
| Explain theory 2. Molecular Mimicry |
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Definition
immune system responds to antigen of a pathogen, but we have self-antigens that have very similar molecular structure
--> so the immune system fights the pathogen, and it cross reacts with self antigens |
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Term
| In the molecular mimicry theory, what is the most common shared molecular structure? |
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Definition
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Term
| What is the main function of the pancreas? |
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Definition
| It secretes digestive enzymes into the digestive tract |
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Term
| What type of cells are in the islet of langerhans in the pancreas? |
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Definition
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Term
| What is the function of the alpha and beta cells? |
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Definition
alpha - mainly structural
beta cells - secrete insulin in response to high glucose levels in the blood (which will take it out and sequester it in cells) |
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Term
| Beta cells interact with ___, they don't secrete anything into the gut. |
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Definition
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Term
| In type 1 diabetes, how are islet beta cells destroyed? |
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Definition
by CD4+ T cell mediated immunity and activated macrophages
and also a bit by CTL killing |
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Term
| Explain the evidence that viral infection may provide a trigger for diabetes. |
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Definition
| A researcher in Calgary says some viruses have antigens on their surface that are similar to ones on beta cells -- when immune system produces antibodies to the viruses, you'll get killing of beta cells. |
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Term
| Type 1 Diabetes susceptibility is linked to _ _ _ genotype |
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Definition
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Term
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Definition
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Term
| What are the 3 regions for MHC Class II |
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Definition
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Term
| What are the 3 regions for Class I MHC? |
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Definition
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Term
| Because you get one DP (for example) from your mom, one from your dad... in total how many do you have? |
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Definition
6 regions so...
12 in total! |
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Term
| Which region of HLA is the main culprit for Type 1 Diabetes? |
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Definition
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Term
| Which types of DR in particular are associated with elevated risk of diabetes? |
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Definition
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Term
| True or false: If your dad is DR3, DR7 and your mom is DR4, DR5 all children will get diabetes. |
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Definition
NO!!
- first of all, not every child will be DR3. DR4
- second, you can be DR3, DR4 and not get diabetes --> it just makes you have higher susceptibility |
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Term
| What other types of HLA may bed linked to risk of Diabetes but to a lesser extent than DR |
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Definition
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Term
| Where are differences between DR3 and DR4? |
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Definition
| in the AA sequence --> in the alpha helices and beta pleated sheets |
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Term
| Why does everyone have slightly different alpha helices and beta pleated sheets? |
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Definition
| Because as a population, we want to be able to respond to any kind of antigens |
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Term
| Where do islet cells go when you put them into the portal vein? |
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Definition
Into the liver! (not the pancreas)
there they lodge into the liver sinusoids and produce insulin |
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Term
| What is the problem with Islet cell transplantion? |
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Definition
| The immune response that killed the original islet cells will kill these ones too. --> have to be on life long immunosuppression |
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Term
| Explain a kidney pancreas transplant. |
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Definition
One result of diabetes may be diabetic nephropathy - so need to get a kidney transplant. Since they will have to be on immunosuppression for their whole life, they can also transplant a pancreas!!
KP transplant --> both organs survive and so do the islet cells |
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Term
| Because the main function of the pancreas is to secrete stuff into the bowel, when doing KP transplant, you must also transplant some ___. |
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Definition
duodenum (as an out for the digestive enzymes)
hook it up either to the bladder (pee out) or the bowel, small intestine (secreted into the bowel and go out in normal way) |
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Term
| What is one clinical presentation of Systemic Lupus Erythematosus |
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Definition
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Term
| What are the main complications of Systemic Lupus? |
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Definition
- problems with:
- lungs
- lupus arthritis (painful, debilitating)
- lupus nephritis (can be fatal)
- rashes |
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Term
| Why was lupus called the great imposter? |
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Definition
| because it was difficult to diagnose |
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Term
| What is the female to male ratio for lupus? |
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Definition
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Term
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Definition
production of auto-antibodies that are mostly directed at the nucleus
--> anti-histone antibodies are made |
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Term
| In lupus, where do you get immune complex deposition? |
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Definition
kidney (glomerulonephritis)
inflamed joints (lupus arthritis)
deposition in the skin and lungs |
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Term
| Where does filtration occur in the kidney? |
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Definition
| glomeruli which are in the cortex |
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Term
| Why is the artery entering the glomeruli much bigger than the tubules that come out? |
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Definition
| it increases pressure!! want that b/c you want to push fluid out |
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Term
| Multiple sclerosis is a disease of the __ __. |
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Definition
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Term
| What is the result of having damaged myelin sheath? |
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Definition
myelin helps signals to propagate more quickly
and protects... so without it can get damage to the nerve |
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Term
| Where in the body can you get MS damage? |
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Definition
| in the brain or spinal cord |
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Term
| What immune cells are involved in MS? |
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Definition
- Th1 cells
- macrophages mediate tissue damage
- major antigen is myelin basic protein |
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Term
| What drugs are used for treatment of MS? |
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Definition
- Anti-proliferatives
- Corticosteroids
- Immunosuppression
- Neuropathic pain
- anti-depressives
- Vein liberation (but this doesn't actually work) |
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Term
| Rheumatoid arthritis is characterized by... (2) |
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Definition
| bone destruction and bone displacement |
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Term
| Briefly describe the differences in presentation of rheumatoid arthritis vs osteoarthritis |
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Definition
Rheumatoid -- sitffness in the morning but improvements on use of the joints, bilateral
Osteo--> using joint makes it worse, often unilateral |
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Term
| Is rheumatoid arthritis more common in men or women? |
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Definition
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Term
| What causes Rheumatoid Arthritis? |
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Definition
- Production of auto-antibodies
- get immune complex deposition in the joints causing inflammation |
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Term
| What causes inflammation in rheumatoid arthritis? |
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Definition
-Th1 mediated macrophage activation
-in the late stages -- osteoclasts
- TNF plays a critical role in maintaining the inflammation |
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Term
| Explain rheumatoid factor. |
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Definition
| Not everyone with rheumatoid factor has rheumatoid arthritis and vice versa |
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Term
| How do we treat rheumatoid arthritis? |
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Definition
NSAIDS
Corticosteroids
immunosuppressive drugs
Immune modulation |
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Term
| Graves disease is characterized by ___ ___. |
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Definition
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Term
| What leads to Grave's disease? |
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Definition
| a problem with thyroid hormone production |
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Term
| Explain how thyroid hormone is produced and regulated. |
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Definition
- The pituitary gland secretes thyroid stimulating hormone which acts on the thyroid to induce the release of thyroid hormone
- the pituitary sense the amount of thyroid hormone, and if there is too much it'll stop producing TSH |
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Term
| What goes wrong in the production of thyroid hormone with Grave's disease? |
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Definition
| Autoimmune B cells make agonist antibodies to the TSH receptors that act like TSH and stimulate the production of thyroid hormone. --> get continuous excess thyroid hormone |
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Term
| Myasthenia Gravis is the opposite to ___. |
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Definition
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Term
| Myasthenia gravis is characterized by |
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Definition
| Ptosis - drooping of one eyelid |
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Term
| Grave's disease is an ___ antibody disease whereas Myasthenia Gravis is an ___ antibody disease |
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Definition
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Term
| Normally, ACh binds to ACh receptors and produce a response in the muscles. What happens in myasthenia graves? |
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Definition
| antagonist antibodies bind and therefore block ACh from binding to the receptor so can't get the muscle signal |
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