摘要目的:探讨骨骼肌单器官血管炎患者临床和骨骼肌病理特点、治疗及预后。方法:回顾分析2017年1月至2019年12月中日友好医院确诊的5例骨骼肌单器官血管炎患者的临床和骨骼肌病理特点、治疗及随访情况。结果:5例中男3例，女2例，中位发病年龄36岁。下肢肌肉疼痛和发热发生率分别为100%（5/5）和20%（1/5），白细胞计数（WBC）、红细胞沉降率（ESR）、C反应蛋白（CRP）和血肌酸激酶（CK）升高比例分别为20%（1/5）、100%（5/5）、100%（5/5）和0。4例肌电图检查均未见肌源性损害。5例（100%，5/5）肌肉MRI在T 2加权像（T 2WI）和（或）短时反转恢复（STIR）序列上表现为肌肉内弥漫/斑片状高信号和T 1加权像（T 1WI）上正常信号。5例患者肌肉病理均为非肉芽肿性血管炎，2例伴有管壁纤维素性坏死，未见肌细胞坏死及多核巨细胞或肉芽肿；小动脉受累5例，受累血管分布肌束膜3例（75.0%，3/4），肌筋膜+肌束膜1例（25.0%，1/4）。5例均接受糖皮质激素联合免疫抑制剂治疗。中位随访时间24个月，3例持续缓解，2例复发。 结论:骨骼肌单器官血管炎常出现下肢肌痛、发热，高ESR/CRP而CK不高。确诊需典型肌肉病理和半年以上随访排除系统血管炎骨骼肌受累。糖皮质激素、免疫抑制剂治疗有效。

abstractsObjectives:To delineate clinico-pathological features, treatment and outcome of skeletal muscle single-organ vasculitis (SM-SOV).Methods:The clinico-pathological characteristic of SM-SOV cases treated over 3 years in China-Japan Friendship Hospital were retrospectively analyzed and the data were compared with the cases from the literature.Results:Five patients (2 women and 3 men) with a median age of 36 years were included in this study. The main clinical manifestations were lower limb myalgia (5/5) and fever (1/5). The most frequent laboratory findings included high erythrocyte sedimentation rate (5/5), high C reactive protein (5/5) and leukocytosis (1/5). No elevated creatine kinase (CK) was found in these cases. Four patients received electromyogram examination and none of them showed myogenic injury. On MRI, hyperintense signals in T 2 weighted image (T 2WI) and/or short TI inversion recovery (STIR) and normal unenhanced T 1 weighted image (T 1WI) of one or several leg muscles was founded in all 5 patients. All muscle specimens showed nongranulomatous vasculitis without myonecrosis affecting small sized artery (5/5) in perimysia (75.0%, 3/4) or both perimysia and fascia (25.0%, 1/4). Corticosteroids (5/5) and immunosuppressants (5/5) were the main agents prescribed. With a median follow-up of 24 months, sustained remission was observed in 3 patients, relapses occurred in 2 patients. Conclusion:SM-SOV should be considered for patients with lower limb myalgia, high inflammatory markers and normal/low CK level. The diagnosis of SM-SOV should be applied when there are both histologic evidence of vasculitis and a minimum of 6 months of follow-up surveillance without evidences suggesting extra-muscular involvement. Corticosteroid combined with immunosuppressant is effective.