骨骼肌单器官血管炎临床、影像及病理分析

Clinico-imageologico-pathologic analysis of single-organ skeletal muscle vasculitis

目的探讨骨骼肌单器官血管炎(SoSMV)患者临床、肌肉磁共振成像(MRI)以及肌肉病理特点。方法回顾分析2017年1月至2022年12月中日友好医院确诊的SoSMV患者的临床、肌肉MRI以及骨骼肌病理资料、治疗和转归情况。结果13例中男7例,女6例,中位发病年龄49岁。下肢肌肉疼痛、肌肉肿胀和发热的发生率分别为100.0%、76.9%和53.8%。13例(100.0%)红细胞沉降率和C反应蛋白均升高,7例(53.8%)白细胞计数升高,5例(38.5%)血清C1q循环复合物水平升高。血清肌酸激酶(CK)水平正常4例(30.8%),9例(69.2%)降低。1例(1/9,11.1%)肌电图提示肌源性损害。13例(100%)肌肉MRI在T2加权像和/或T2加权脂肪抑制像上表现为肌肉内弥漫分布、边界不清的条纹状、分支状、小斑片状高信号和T1加权像上的正常/稍低信号。13例患者肌肉病理均为非肉芽肿性血管炎,其中4例(30.8%)为坏死性血管炎,9例(69.2%)为非坏死性血管炎;其中5例(38.5%)可见不同程度的肌细胞变性坏死伴炎症和纤维化,1例(7.7%)可见肌细胞角样萎缩,7例(53.8%)肌纤维大致正常。13例均接受糖皮质激素治疗,其中6例(46.2%)联合免疫抑制剂治疗。中位随访时间36个月,7例(53.8%)持续缓解,6例(46.2%)复发。结论SoSMV常出现下肢肌痛、发热,炎症指标升高,血清CK正常,甚至出现低CK血症。特征性肌肉MRI表现有助于诊断。糖皮质激素、免疫抑制剂治疗有效。

Objective To delineate clinico-imageologico-pathologic features of single-organ skeletal muscle vasculitis(SoSMV).Methods We retrospectively analyzed SoSMV cases observed over a 6-year period from our center.Results We included 13 patients(6 women and 7 men)with a median age of 49 years.The main clinical manifestations were lower limb myalgia(100%),muscle swelling(76.9%)and fever(53.8%).The most frequent laboratory findings include high ESR(100.0%),high CRP(100.0%)and leukocytosis(53.8%).There was no hyper-CK-emia founded.Nine patients underwent EMG examination and 1 showed myogenic injury.On MRI,diffuse,patchy,or ramified hyperintense signals in T2WI and or STIR and normal hypointense in T1WI of one or several leg muscles was founded in all 13 patients.All muscle specimens showed nongranulomatous vasculitis affecting small sized artery.Corticosteroids(100.0%)and immunosuppressants(46.2%)were the main agents prescribed.With a median follow-up of 36 months,sustained remission was observed in 7 patients(53.8%),relapses occurred in 6 patients(46.2%).Conclusion SoSMV should be considered for patients with lower limb myalgia,high inflammatory markers and normal low CK level.Lower extremity MRI has a higher ability to detect vasculitic muscle disease and could constitute a useful complementary examination for SoSMV.Corticosteroid and immunosuppressant are effective.