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22 Cards in this Set
- Front
- Back
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213. Idiopathic Inflammatory myopathies?
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1. Polymyositis
2. Dermatomyositis 3. Childhood onset dermatomyositis- subcutaneous calcifications 4. Myositis associated w/collagen vascular disease 5. Myositis associated w/malignancy 6. Inclusion body myositis |
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214. In whom are Idiopathic inflammatory myopathies more common, men or women?
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a. Females.
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215. Polymyositis?
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a. Polymyositis is used when the condition does not involve the skin (usually occurs in adults)
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216. How is Inclusion Body Myositis an oddball among the idiopathic Inflammatory myopathies?
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a. It affects males more than females.
b. Absence of autoantibodies c. Distal muscle involvement d. Relatively low creatine kinase (CK) |
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217. Prognosis of Inclusion Body Myositis?
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a. Poor prognosis.
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Hypothesized cause of Idiopathic Inflammatory myopathies?
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a. A genetically susceptible individual plus an environmental trigger leads to immune activation, which results in chronic inflammation.
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219. Mechanism of Pathologic changes in muscle with Dermatomyositis vs. polymyositis?
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a. Dermatomyositis: Humoral immune mechanisms
b. Polymyositis: Cell-mediated process. |
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220. Clinical features common to both Polymyositis and dermatomyositis?
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a. Symmetrical proximal muscle weakness that develops subacutely over weeks or several months.
i. The earliest and most severely affected muscle groups are the neck flexors, shoulder girdle, and pelvic girdle muscles. ii. Distal extremity weakness is less frequent and typically less severe. b. Myalgia in 33% of patients c. Dysphagia in up to 30% of patients (involvement of esophageal muscles). |
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221. What features are unique to dermatomyositis?
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a. Heliotrope rash (butterfly)-around eyes, bridge of nose, cheeks
b. Gottron’s papules- papular, erythematous, scaly lesions over the knuckles (MCP, PIP, DIC) c. V sign- rash on the face, neck, anterior chest d. Shawl sign- rash on shoulders, upper back, elbows and knees e. Periungual erythema with telangiectases f. Subcutaneous calcifications in children- can be extremely painful |
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222. Associated findings in both polymyositis and dermatomyositis?
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a. Arthralgias (common)
b. CHF and conduction defects (rare) c. Interstitial lung disease (in minority patients) |
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223. Associated findings in dermatomyositis only?
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a. Vasculitis of the GI tract, kidneys, lungs and eyes (more common in children)
b. There is an increased incidence of malignancy in older adults. c. Once are my myositis is diagnosed, make an effort to uncover an occult malignancy. d. Dermatomyositis associated with malignancy often remits once the tumor is removed! |
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224. In what organs is the increased malignancy with dermatomyositis?
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a. Lung
b. Breast c. Ovary d. GI tract e. Myeloproliferative disorders |
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225. What standard lab values are elevated with dermatomyositis and polymyositis?
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a. CK level: significantly elevated
b. LDH c. Aldolase d. AST/ALT e. ANA in over 50% |
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226. What autoantibody is associated with dermatomyositis and polymyositis?
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a. Anti-synthetase antibodies (anti-Jo-1 antibodies)
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227. Significance of Anti-synthetase antibodies (anti-Jo-1 antibodies)?
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1. Associated with abrupt onset of fever, cracked hands, Raynaud's phenomenon, Interstitial lung disease, and arthritis.
2. Does not respond well to therapy |
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228. What is the significance of anti-signal recognition particle with dermatomyositis and polymyositis?
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a. Cardiac manifestations (common)
b. worst prognosis of all subsets |
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229. What is the significance of anti-Mi antibodies with dermatomyositis and polymyositis?
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a. Better prognosis.
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230. With dermatomyositis and polymyositis, with what does the CK level correspond?
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a. The degree of muscle necrosis, so one can monitor the disease severity.
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231. Value EMG with dermatomyositis and polymyositis?
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a. Abnormal in 90% of patients
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232. What does a muscle biopsy show with all three (Dermatomyositis, Polymyositis and Inclusion body myositis)?
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a. Inflammation and muscle fiber fibrosis in all three.
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233. Where is the inflammation and muscle fibre fibrosis unique to dermatomyositis only?
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a. Perivascular and perimysial inflammation.
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234. Where is the inflammation and muscle fibre fibrosis unique to Polymyositis and Inclusion body myositis?
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a. Endomysial
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