包涵体肌炎的临床与病理特点(附2例报告)

Clinical and pathological features of inclusion body myositis:two cases report

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 （IBM）. Methods The clinical data of the two cases with IBM were collected and the muscle biopsy was done for routine and histochemical staining. Light and electron microscopic studies were also made. Results Two patients had onset at 41 and 54 years old,respectively. They had an onset with lower limbs weakness. The distal weakness was greater than proximal weakness, combined with involving upper limb gradually. The levels of muscle enzymes in the serum were increased. Electromyography showed myopathic damage. Light microscopic examination showed muscle fibers with rimmed vacuoles. There was a few necrotic fibers with inflammatory cells infiltration. Under the electron microscope, amyloid fibrils or tubulofilament inclusion in the cytoplasms were found. Conclusion Clinical feature of IBM is nonspecific, muscle biopsy plays an important role in the diagnosis of IBM.