骨骼肌单器官血管炎五例临床病理分析

Clinico-pathological and follow-up analysis of 5 skeletal muscle single-organ vasculitis cases

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T2加权像(T2WI)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T1加权像(T1WI)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。

Objectives 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 T2 weighted image(T2WI)and/or short TI inversion recovery(STIR)and normal unenhanced T1 weighted image(T1WI)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.