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Flashcards in Autoimmune disorders from pathoma Deck (20):

What are the primary mechanisms that the body uses to prevent autoimmunity?

negative selection of T cells in the thymus or bone marrow via apoptosis
anergy in the periphery- get rid of self-reactive cells if they don't get a second signal


SLE: What kind of hypersensitivity? Demographics?

this is a type II (cytotoxic) and a type III (antigen-antibody complex) mediated disorder
most common in women, esp. African Americans


Clinical features of SLE

they will have fever and weight loss
RASH OR PAIN mnemonic:
rash (malar or discoid)
serositis: pleuritis or pericarditis
hematologic disturbances like anemia, thrombocytopenia, or leukopenia d/t autoantibodies against cell surface proteins
oral ulcers or nasopharyngeal ulcers
renal disease; raynauds
antinuclear antibodies
neurologic disorders like seizures or psychosis


Common causes of death in SLE

CV disease, infections, and renal disease


What is the most common renal lesion seen in pts with SLE?

diffuse proliferative glomerulonephritis


What are important CV findings in SLE?

Libman-Sacks endocarditis: small, sterile deposits on BOTH sides of the mitral valve. these are deposition of ab-antigen complexes


What are the important autoantibodies in SLE?

ANA (sensitive, but not specific), and anti-dsDNA antibodies (very specific)
(don't memorize, but if you're going crazy, remember that anti-Smith is an ANA specific for lupus)


What characterizes drug-induced SLE? What are common offenders and what is the treatment?

antihistone antibody
common offenders: isonizid, hydralazine, and procainamide
usually resolves with removal of the drug


What is antiphospholipid syndrome? Associations, manifestations, relevant abs, tx

syndrome that may be associated with SLE (30% of cases)
characterized by autoantibody against proteins bound to phospholipids
most commonly, look at anticardiolipin and lupus anticoagulant
this disease casues arterial and venous thrombosis, including DVTs, hepatic vein thrombosis (Budd-Chiari), placental thrombosis, and stroke
requires lifelong anticoagulant therapy. remember that warfarin is contraindicated in pregnancy


What do I need to know about anticardiolipin antibodies and lupus anticoagulant?

they can lead to false-positives on syphilis tests and falsely elevated PTT lab studies, respectively.
note that elevated PTT is paradoxical, because these antibodies actually increase the risk of arteriovenous thromboembolism)


What is Sjogren syndrome? What type of hypersensitivity rxn?

autoimmune destruction of the lacrimal and salivary glands
this is a lymphocyte-mediated type IV hypersensitivity rxn with fibrosis


Presentation of Sjogren and relevant autoantibodies

presents as dry eyes (keratoconjunctivitis), dry mouth (xerostomia), and recurrent dental caries in older woman (50-60 yrs)
characterized by ANA and anti-ribonucleoprotien antibodies (anti-SSa/Ro and anti-SSB/La)


What diseases are associated with Sjogren syndrome

other autoimmune diseases, esp. rheumatoid arthritis
incr. risk for B cell (marginal zone) lymphoma, which presents as unilateral enlargement of the parotid gland


What is scleroderma? Subcategories?

autoimmune disease characterized by activation of fibroblasts and deposition of collagen
divided into localized and systemic scleroderma


What should I know about localized scleroderma?

involves skin only
most common subtype is called morphea
highly associated with antibodies to DNA topoisomerase II


What does systemic scleroderma involve? Subcategories?

skin and visceral organs
classified as limited or diffuse


What is limited systemic scleroderma?

limited areas of skin (hands, face, and neck with late visceral involvement. CREST syndrome: calcinosis/anti-centromere antibodies, raynaud, esophageal dysmotility, sclerodactyly, and telangiectasias of the skin


What is diffuse systemic scleroderma?

involves skin and any visceral organ. most commonly GI tract, lungs, CV, and kidneys. lungs are the most common cause of death. ascociated with anti-Scl-70 antibody, which is an anti-DNA topoisomerase I antbody


Tx for SLE

NSAIDs, steroids, immunosuppressants, hydroxychloroquine


What is mixed connective tissue disease?

autoimmune mediated tissue damage with mixed features of SLE, systemic sclerosis, and polymyositis
charachterized by serum antibodies against U1 ribonucleoprotein

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