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53 Cards in this Set
- Front
- Back
Two basic principle of autoimmune disorders. |
(1) Characterized by immune-mediated damage of tissues.
(2) Involves loss of self-tolerance |
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What is the prevalence of autoimmune disorders?
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1% in the USA. More common in women; classically affects women of childbearing age.
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What is the etiology of autoimmune disorders?
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Etiology is likely an environmental trigger in genetically susceptible individuals (increased incidence in twins and associated with certain HLA subtypes).
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What mediates the damage in SLE?
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Autoantibodies against the host damage multiple tissues via type II HST (cytotoxic) and type III (Ag-Ab complexes) HST.
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SLE is more common in ________ (men/women), especially ___________.
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women; african-american women.
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What are clinical features of SLE?
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• Fever, weight loss (chronic inflammation)
• Malar 'butterfly' rash, especially upon sun exposure • Arthritis • Serositis (pleura, pericardium) • Inflammation of brain; CNS psychosis |
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The most common cause of death in lupus patients is _________. Most commonly due to what pattern of injury? Aside from this, a common cause of death is ________.
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renal damage; diffuse proliferative glomerulonephritis; infection.
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True or false: SLE can involve the heart.
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True. All three layers. Can produce a characteristic Libman-Sacks endocarditi. One of the key findings is the presence of vegetations on BOTH sides of the heart valve involved.
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True or false: In SLE there may be autoantibodies to formed blood elements.
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True. There may be formed antibodies against RBCs, PLTs, WBCs.
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This autoantibody is seen in CREST syndrome.
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C is for Calcinosis and anti-Centromere antibody
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This autoantibody is seen in celiac disease.
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(1) Antiendomysial IgA
(2) Antigliadin IgA (3) Anti-tissue transglutaminase IgA |
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This autoantibody is seen in drug-induced lupus.
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Anti-histone
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This antibody is seen in DM type I.
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Anti-insulin
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This autoantibody is seen in Hashimoto's.
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Antimicrosomal antibody
Antithyroglobulin |
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This autoantibody is seen in SLE.
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(1) Anti-Smith (Sm)
(2) ANA (3) Anti-dsDNA |
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This autoantibody is seen in primary biliary cirrhosis.
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Antimitochondrial antibody
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This autoantibody is seen in Sjögrens.
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Anti-SS-A (Ro)
Anti-SS-B (La) |
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This antibody is seen in microscopic polyangiitis.
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Antimyeloperoxidase antibody.
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This autoantibody is seen in mixed connective tissue disease.
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Anti-ribonucleoprotein.
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This autoantibody is seen in Wegener's granulomatosis.
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(1) C-ANCA
(2) Antiproteinase 3 antibody |
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This autoantibody is seen in systemic sclerosis.
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(1) Anti-topoisomerase
(2) ANA |
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This autoantibody is seen in dermatomyositis.
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ANA
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Anti-dsDNA is highly ______ (sensitive/specific) for SLE.
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specific
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ANA is ________ (sensitive/specific) for SLE.
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sensitive
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These drugs are common causes of drug-induced SLE.
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(1) Hydralazine
(2) Procainamide (3) Isoniazid |
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What is the treatment of drug-induced SLE?
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Remove drug. It usually results in remission.
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This syndrome is associated with SLE in _____% of cases.
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antiphospholipid syndrome; 30%
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Antiphospholipid syndrome is associated with what?
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Autoantibody against proteins bound to phospholipids.
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In antiphospholipid syndrome the MC antibodies are [...]
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Anticardiolipin and lupus anticoagulant
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The presence of autoantibodies in antiphospholipid syndrome could interfere with what? |
Lead to false-positive syphilis test and falsely-elevated PTT lab studies, respectively
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Why is lupus anticoagulant called an anticoagulant.
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It produces a falsely elevated PTT.
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Lupus anticoagulant results in _______________. Treatment is?
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Results in arterial and venous thrombosis including deep venous thrombosis, hepatic vein thrombosis, placental thrombosis (recurrent pregnancy loss), and
stroke. Lifelong anticoagulation. |
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What is Sjögren syndrome? What type of damage is it?
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Autoimmune destruction of lacrimal and salivary glands. Lymphocyte-mediated damage (type IV hypersensitivity) with fibrosis.
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Sjögren clinically presents as?
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Classically presents as dry eyes (keratoconjunctivitis), dry mouth (xerostomia), and recurrent dental carries in an older woman (50-60 years)—"Can't chew a cracker, dirt in my eyes".
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A woman has bilaterally enlarged parotid glands with dry eyes. Treatment is begun and parotid glands shrink back to normal. A few years later, the left parotid gland has again swelled up. What is going on?
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Sjögrens syndrome has an increased risk for B-cell (marginal zone) lymphoma.
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Autoimmune disorders are often associated with _________________.
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other autoimmune disorders
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A woman with dry eyes and mouth develops nodules on her forearm close to her elbow. What is going on?
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Sjögren is associated with other autoimmune diseases, especially Rheumatoid arthritis.
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Another word for scleroderma is ___________.
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systemic sclerosis
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What is scleroderma?
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Autoimmune tissue damage with activation of fibroblasts and deposition of collagen (fibrosis) . Primarily targets the skin (scleroderma), gastrointestinal tract, lungs, and kidneys.
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A woman has the following sign. This is the MC initial sign of what?
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Raynaud's phenomenon, MC initial sign of systemic sclerosis.
Sequential color changes (white to blue to red) caused by digital vessel vasculitis and fibrosis |
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Describe the two types of systemic sclerosis.
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(1) Diffuse type (skin and early visceral involvement). Almost any organ can be involved; esophagus is commonly affected, resulting in disordered motility (dysphagia for solids and liquids).
(2) Localized type (local skin and late visceral involvement). Prototype is CREST syndrome: Calcinosis/anti-Centromere antibodies, Raynaud phenomenon. Esophageal dysmotility, Sclerodactyly, and Telangiectasias of the skin. |
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What is MCTD?
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Mixed connective tissue disease.
Autoimmune-mediated tissue damage with mixed features of SLE, systemic sclerosis, and polymyositis. Characterized by serum antibodies against U1 ribonucleoprotein |
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In systemic sclerosis, the MCC of death is ____________.
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respiratory failure
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What is your first clinical suspicion?
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The MC cardiac finding in SLE is ___________________.
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fibrinous pericarditis with effusion
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A woman with SLE delivers a newborn. What complications in the newborn could you expect to see?
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Complete heart block in newborns
• Caused by IgG anti-SS-A (Ro) antiboilies crossing the placenta |
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A woman with SLE has recurrent spontaneous abortions. The doctor states that this is unrelated to her condition. Is he right?
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He is wrong. Recurrent spontaneous abortions can be caused by antiphospholipid antibodies.
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Would serum complement be high or low in SLE?
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Low due to complement activation due to immune complexes.
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In the pathogenesis of SLE, genetic links appear to be located on chromosome __.
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6
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Provide five examples of environmental factors that may be important in exacerbating or triggering SLE.
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(1) Infections (virus/bacteria)
(2) UV (3) Estrogen (4) Medications (5) Extreme stress They destroy cells, leading to Abs directed against various nuclear antigens. |
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A serum __________ is the most useful screening test for autoimmune diseases.
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ANA
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This type of transplant has the best allograft survival rate. It is associated with what? |
cornea; danger of transmission of Creutzfeldt-Jakob
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The fetus is an ___________ that is not rejected by the mother. What tissue may prevent maternal T cells from entering the fetus?
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allograft
trophoblastic tissue |