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34 Cards in this Set

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