包涵体肌炎的临床与肌肉病理特征

INCLUSION BODY MYOSITIS: CLINICAL AND MYOPATHOLOGICAL FEATURES

包涵体肌炎在国内鲜见，兹对其临床与肌肉病理特征作一复习。报告一例包涵体肌炎老年男性患者，隐袭起病，病情缓慢进展长达２３年，临床表现以吞咽困难为首发症状，７年后出现以双侧股四头肌为主的、对称性近端肌无力和肌萎缩，胸锁乳突肌、腹肌也受累，不伴有肌痛。有类风湿性关节炎病史。肌电图示肌原性受损。血沉、肌酸激酶、免疫球蛋白Ｇ呈轻中度升高，类风湿因子阳性。肌肉活检表现为：非坏死肌纤维的单核细胞浸润，特征性的边缘着色性空泡，电镜下发现有确诊意义的胞浆内包涵体，内含大量丝状物，确认为包涵体肌炎。提示在遇有上述特征性改变的患者应想到本病。

Inclusion body myositis has been recognized as a major form of idiopathic inflammatory myopathy. An old male patient with insidious onset and slowly progressive muscular weakness and artrophy has been reported in this article. The duration of symptom before biopsy was 23 years. The first symptom was dysphagia, and muscular weakness developed seven years later. Muscular atrophy was predominant symmetrically and proximally, particularly the quadriceps femoris muscles. Cervical and abdominal muscles were also affected. Myalgia was absent. Electromyogrophy showed myopathic alterations. Erythrocyte sedimentation rate, creatine kinase, immunoglobulins G increased slightly or moderately. Rheumatoid factor was positive, and he had been diagnosed as having rheumatoid arthritis for 23 years. Inclusion body myositis was ultimately diagnosed based on the muscle biopsy which showed mononuclear cell invasion of nonnecrotic muscle fibers, the characteristic rimmed vacuoles in cryostat sections and cytoplasmic inclusion bodies consisted of plenty of tubulofilaments by electron microscope.