Idiopathic Inflammatory Myopathies (IIM): Classification, Clinical Features, Diagnosis, and Management
Idiopathic inflammatory myopathies (IIM) are a group of rare systemic diseases characterized by progressive weakness due to chronic skeletal muscle inflammation. Major subtypes include dermatomyositis (DM), overlap myositis (OM), immune-mediated necrotizing myopathy (IMNM), inclusion-body myositis (IBM), and polymyositis (PM). Juvenile forms (JIIM) occur before age 18. Typical manifestations include symmetric proximal muscle weakness (difficulty reaching overhead, climbing stairs, standing up), cutaneous features (Gottron papules, heliotrope rash, shawl sign), and systemic involvement (interstitial lung disease, malignancy risk, cardiac manifestations). Diagnosis is supported by elevated muscle enzymes (creatine kinase, aldolase), electromyography, and muscle biopsy. Management includes supportive therapy (physical/occupational therapy) and immunosuppression (glucocorticoids, methotrexate, azathioprine), except for IBM where treatment is supportive only.
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