摘要：

目的: 总结抗Ku或抗PM-Scl抗体阳性硬化性肌炎患者临床表现及骨骼肌组织病理学特征。方法与结果: 回顾分析2010年1月至2023年3月北京大学第一医院明确诊断的70例硬化性肌炎患者临床资料，中位发病年龄36.00(20.75，51.25)岁，77.14%(54/70)以肌无力首发，且以四肢近端肌无力为主[74.29%(52/70)]，多呈轻至中度[77.14%(54/70)]；52.86%(37/70)病程中伴疲劳感、皮肤受累等全身症状；血清肌酸激酶中位值为2775.00(995.50，4995.75)U/L，32.86%(23/70)抗Ku抗体阳性、65.71%(46/70)抗PM-Scl抗体阳性、1.43%(1/70)二者皆阳性，27.14%(19/70)同时合并其他肌炎抗体阳性。组织病理改变以炎症性[41.43%(29/70)]或坏死性[24.29%(17/70)]肌病样改变为主，呈肌纤维萎缩[91.43%(64/70)]、坏死[68.57%(48/70)]和再生[85.71%(60/70)]。抗Ku抗体阳性与抗PM -Scl抗体阳性患者症状与体征以及骨骼肌组织病理学特征比较，抗Ku抗体阳性患者女性(χ²=4.381，P=0.036)和补体C5b9沉积(χ²=4.725，P=0.030)比例高于抗PM-Scl抗体阳性患者。结论: 伴抗Ku或抗PM-Scl抗体阳性硬化性肌炎患者多于中青年期发病，表现为四肢近端轻至中度肌无力，骨骼肌炎症性或坏死性肌病样损害为特征性病理改变；与抗PM-Scl抗体阳性患者相比，抗Ku抗体阳性硬化性肌炎患者以女性居多且骨骼肌补体沉积更为明显。

关键词: 肌炎, 硬皮病, 系统性, 抗体, 肌无力, 病理学, 免疫组织化学

Abstract:

Objective: To summarize the clinical manifestations and histopathological characteristics of skeletal muscle in scleromyositis patients with positive anti-Ku or anti- PM -Scl antibodies. Methods and Results: Retrospective collection of clinical data from 70 scleromyositis patients diagnosed at Peking University First Hospital from January 2010 to March 2023, analysis showed the average onset age of patients was 36.00 (20.75, 51.25) years old. The initial symptoms of 77.14% (54/70) patients was mainly in the proximal limbs myasthenia [74.29% (52/70)], mild to moderate [77.14% (54/70)], and 52.86% (37/70) patients had systemic symptoms such as fatigue or skin involvement during the course of the disease. The serum creatine kinase (CK) level was 2775.00 (995.50, 4995.75) U/L. About 32.86% (23/70) were positive for anti - Ku antibody, 65.71% (46/70) were positive for anti - PM - Scl antibody, 1.43% (1/70) were both positive, and 27.14% (19/70) could also had increased level of other myositis antibodies. The main histopathological changes were inflammatory [41.43% (29/70)] or necrotic [24.29% (17/70)] myopathic pathological changes, presenting as muscle fiber atrophy [91.43% (64/70)], necrosis [68.57% (48/70)] or regeneration [85.71% (60/70)]. Comparison of symptoms, signs and histopathological characteristics of skeletal muscle between patients with positive anti - Ku antibody and with positive anti - PM - Scl antibody, female proportion (χ²=4.381, P=0.036) and C5b9 deposition damage (χ²=4.725, P=0.030) were higher in the former than those in the latter. Conclusions: Scleromyositis patients with positive anti-Ku or anti-PM -Scl antibodies mostly started in young and middle age, mainly manifesting as mild to moderate myasthenia in the proximal limbs, and skeletal muscle inflammatory or necrotic myopathic lesions as characteristic pathological changes. Compared with positive anti - PM - Scl antibody patients, positive anti - Ku antibody scleromyositis patients were mainly female, and skeletal muscle complement deposition was more notable.

Key words: Myositis, Scleroderma, systemic, Antibodies, Muscle weakness, Pathology, Immunohistochemistry