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34 Cards in this Set
- Front
- Back
Onset around 50(rare in children), female predominance, hx of chemical exposure, pentazosine, toxic oil, L-tryptophan
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Scleroderma
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What are the localized forms of scleroderma?
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Linea Sabre and Morphea(erythematous periphery with central shininess)
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CREST syndrome?
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Calcinosis, Reynauds, Esophageal dysmotility, Sclerodactyly, Teleangiectasias, usually with a positive anticentromere Ab and also pulmonary vasculopathy
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Symptoms of diffuse scleroderma?
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Reynauds, puffy fingers, fibrotic skin, ILD, Renal Crisis (tx with ACE I) Dermal Fibrosis
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When is Reynauds indicative of Scleroderma?
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Vasoconstriction which causes ulcerations and autoamputation of the ischemic tissue
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What are GI symptoms of Scleroderma?
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Esophageal dysmotility, watermelon stomach, colonic diverticulitis
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Which type of Scleroderma is seen with Renal Disease
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Diffuse Scleroderma, not crest disease
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Which meds are used to treat scleroderma
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methotrexate, prednisone, cytoxan, cellcept. Artificial tears, Ace I, Proton Pump inhibitors can be used for symptomatic treatment.
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Which antibodies are positive in scleroderma?
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ANA, Anticentromere in localized Scleroderma, Scl70 in 40% of diffuse
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Most common inflammatory myopathy?
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Polymyositis, followed by dermatomyositis, childhood dermatomyositis, overlap, malignancy.
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Pathogenesis of polymyositis
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mainly cellular mediated process resulting in infiltration of cytotoxic CD8+ cells in the endomysium
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Pathogenesis of dermatomyositis
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characterized by perivascular CD4+ T cells &B cell infiltration along with deposition of late complement components (perimysium)
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MSK manifestations of derma. and polymyo?
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Insidious proximal muscle weakness
Bulbar muscle weakness Heliotrope rash of dermatomyositis Gottron’s papules Raynaud’s Arthritis |
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What are the extramuscular manifestations of inflammatory myopathies?
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fever, fatigue, aspiration, ILD, calcinosis, heliotrope rash, shawl sign rash and gottrens papules in Dermatomyositis,
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Whcih antibodies indicate a good prognosis for dermatomyositis/polymyositis?
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Anti-Mi2
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Which antibody indicates a worse prognosis for dermatomyositis/polymyositis
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Anti-Jo1
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How are dermatomyositis and polymyositis diagnosed?
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gold standard: muscle biopsy, most patients have positive EMG
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What are some non causes for noninflammatory myopathies?
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Hypokalemia, Hypothyroidism, infection, colchicine, PCN, chloroquine, lovastatin, AZT, corticosteroids
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anti-Jo1, anti-SRP, dysphagia, myocardial involvement are indicative of what kind of prognosis for inflammatory myopathies?
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poor outlook
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anti-MI2, DM, myositis in overlap, inclusion body myositis are indicative of what kind of prognosis for inflammatory myopathies?
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good outlook
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How are inflammatory myopathies treated?
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Prednisone 1mg/kg/day
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Systemic disease most frequently manifested by dry eyes,dry mouth & arthritis
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Sjogren's Disease
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Pathogenesis of Sjogren's disease?
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autoimmune destruction of exocrine glands
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What is the triad of criteria for Sjogren's?
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Keratoconjunctivitis Sicca, Xerostomia, Connective Tissue Disease
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Which antibodies are positive in Sjogrens?
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Positive ANA, Positive SS-A, Positive SS-B, and positive RF
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How is Sjogren's diagnosed?
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Could have enlarged Parotid glands. Salivary Gland Biopsy, which shows diffuse CD4+ lymphocytic foci. Also could have increased ESR, hyperglobulinemia, +RF, +ANA, +SSB
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How is Sjogren's treated?
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Treat the symptoms with artificial tears and saliva, hydration, salagen and restasis. Treat systemic symptoms with Plaquenil, Methotrexate, Imuran
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Systemic disease most frequently manifested by dry eyes,dry mouth & arthritis
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Sjogren's Disease
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Pathogenesis of Sjogren's disease?
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autoimmune destruction of exocrine glands
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What is the triad of criteria for Sjogren's?
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Keratoconjunctivitis Sicca, Xerostomia, Connective Tissue Disease
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Which antibodies are positive in Sjogrens?
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Positive ANA, Positive SS-A, Positive SS-B, and positive RF
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How is Sjogren's diagnosed?
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Could have enlarged Parotid glands. Salivary Gland Biopsy, which shows diffuse CD4+ lymphocytic foci. Also could have increased ESR, hyperglobulinemia, +RF, +ANA, +SSB
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How is Sjogren's treated?
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Treat the symptoms with artificial tears and saliva, hydration, salagen and restasis. Treat systemic symptoms with Plaquenil, Methotrexate, Imuran
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What are the systemic effects of Sjogren's?
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Distal RTA, neurologic manifestations, lymphoma, pulmonary disease, joint symptoms
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