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目的探讨包涵体肌炎的临床与病理特点。方法对2例包涵体肌炎患者的临床表现、肌肉组织化学、酶组织化学和超微结构等资料进行分析。结果本组2例患者分别于41岁及54岁发病,均以双下肢无力起病,远端重于近端,并逐渐向上肢发展;血清肌酶轻～中度升高;肌电图示肌源性损害;肌肉活检光镜下主要表现为肌纤维内出现镶边空泡,少数变性坏死纤维,伴炎性细胞浸润。电镜观察证实肌浆内有大量涡轮状髓样小体及管状细丝包涵体。结论包涵体肌炎临床表现缺乏特异性,肌肉病理学检查是诊断包涵体肌炎的重要手段。
Objective To investigate the clinical and pathological features of inclusion body myositis. Methods The clinical manifestations, muscle histochemistry, enzyme histochemistry and ultrastructure of 2 cases with inclusion body myositis were analyzed. Results The two patients in this group were 41 and 54 years old respectively. Both of them presented with weakness of both lower extremities, distal to the proximal end, and gradually developed to the upper extremity. Serum muscle enzymes were slightly to moderately elevated; Source damage; muscular biopsy under the main performance of the muscle fibers appear striped cavitation, a small number of degenerative and necrotic fibers, with inflammatory cell infiltration. Electron microscopy confirmed a large number of sarcolemma-like medullary bodies and tubular filament inclusions. Conclusion The clinical manifestation of inclusion body myositis lacks specificity. Muscle pathology is an important method to diagnose inclusion body myositis.