Inclusion body myositis. Observations in 40 patients.

摘要: SUMMARY Inclusion body myositis (IBM) was suspected on light microscopic grounds in 48 of 170 consecutive patients with inflammatory myopathies. One or more vacuoles containing membranous material, groups atrophic fibres, and an autoaggressive endomysial exudate occurred 100, 96 92% the muscle specimens All three these features were present 88% Electron microscopy confirmed presence filamentous inclusions 40 43 The are typically near a minimum vacuolated fibres must be scrutinized to detect them confidence. There is no electromyographic pattern that can reliably distinguish IBM from other myopathies typical clinical diagnosed by histological criteria as were: insidious onset after age 50 yrs painless, proximal lower extremity weakness, slow but relentless progression selectively severe involvement quadriceps, iliopsoas, tibialis anterior, biceps triceps muscles; relatively early depression knee reflexes; normal mildly elevated serum creatine kinase level male : female ratio 3 1. Distal weakness about 50%, only 35% it great greater than weakness. Significant associated illnesses include autoimmune disorders (15%), diabetes melhtus (20%), diffuse peripheral neuropathy (18%). Prednisone treatment at dose levels frequently effective polymyositis failed prevent disease those observed for 2 years. Our findings support notion distinct entity which set pathological constellation findings.