包涵体肌炎的临床和病理分析

目的探讨包涵体肌炎的临床和病理特点。方法分析20例包涵体肌炎患者的临床、骨骼肌活检组织化学染色及电镜病理特点。结果骨骼肌活检组织化学染色病理特点：肌周膜、肌内膜、血管周围炎性细胞浸润,肌纤维大小不等,散在变性、坏死和再生肌纤维,结缔组织增生,肌纤维内可见边缘空泡,空泡周边淀粉样物质沉积,可伴破碎红纤维、肌纤维类型或分布异常。电镜：肌原纤维结构紊乱,Z线排列不整或消失,肌浆包涵体内可见大量髓磷脂小体和吞噬空泡聚集,周边可见脂滴、糖原颗粒。结论包涵体肌炎临床诊断、鉴别诊断困难,骨骼肌活检病理分析是确诊本病的可靠标准。

A clinical and pathological analysis of inclusion body myositis

OBJECTIVE: To study the clinical and pathological characteristics of inclusion body myositis (IBM). METHODS: Comprehensive analysis of the characteristics of the clinical, laboratory and pathological of 20 patients with IBM was carried out. RESULTS: Pathological features of biopsied skeletal muscle, inflammatory cells infiltrated in the perimysium, endomysium or around the blood vessels. Muscle fibers in different size, degenerating, necrotic and regenerating fibers scattered, with connective tissue elements increased. Rimmed vacuoles located in the muscles, with amyloid substance deposited around it. Ragged red fibers (RRF), abnormal distribution and types of muscle fibers. Electronmicroscopically, structure of myofibrils disordered, and Z line derangement or dismissed. In the inclusion bodies of sarcoplasma, many myelin bodies and phagocytic vacuoles aggregated, around with lipid drops and glycogen particle. CONCLUSIONS: It is difficult to diagnose IBM with clinical features, while skeletal muscle biopsy and pathological analysis is a trustworthy measure for the definite diagnosis of IBM.