中-小血管炎累及骨骼肌病例临床病理分析及文献复习  

Clinicopathological analysis of skeletal muscle involvement in medium-small vessel vasculitis and literature review

作　　者：李嗣钊 江薇[2] 李文丽[2] 卢昕[2] 王国春[1] LI Si-zhao;JIANG Wei;LI Wen-li(Graduate School,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医学院研究生院,北京100730 [2]中日友好医院风湿免疫科,北京100029

出　　处：《中日友好医院学报》2021年第3期131-134,F0002,共5页Journal of China-Japan Friendship Hospital

摘　　要：目的:探讨中-小血管炎骨骼肌受累患者的骨骼肌病理特征、治疗及预后。方法:总结2017年1月~2019年12月中日友好医院确诊的3例和近20年文献报道的18例中-小血管炎骨骼肌受累患者临床和骨骼肌病理特点、治疗及随访情况。结果:21例中男11例、女10例;中位发病年龄63岁。肌痛、肌无力、横纹肌溶解和发热的发生率分别为100%、57.14%、14.29%和47.62%。皮肤(42.86%)和肾脏(33.33%)受累是最常见的肌肉外表现。8例(47.06%,n=17)以肌肉受累为首发表现。肌酸激酶(CK)升高和ANCA阳性比例为57.14%和52.38%。肌肉病理:66.67%为坏死性血管炎,33.33%为非坏死性血管炎,19.05%见多核巨细胞或肉芽肿。8例(50%,n=16)见肌纤维坏死,4例(25%,n=16)见肌纤维失神经改变,4例(25%,n=16)未见肌细胞萎缩及坏死。21例均接受糖皮质激素治疗,18例(85.71%)联合免疫抑制剂/利妥昔单抗。中位随访时间12个月,80.95%持续缓解,19.05%复发。结论:中-小血管炎骨骼肌受累常出现肌痛、肌无力,皮肤、肾脏受累等肌肉外表现及发热,可伴CK升高,典型肌肉病理可确诊。糖皮质激素、免疫抑制剂治疗有效。Objective:To delineate clinicopathological features,treatment and outcome of skeletal muscle involvement in medium-small vessel vasculitis(SM-MSVV).Methods:We retrospectively analyzed the clinicopathological characteristics of SM-MSVV cases treated over 3 years in China-Japan Friendship Hospital and reviewed the literature in this paper.Results:We included 21 patients(10 women and 11 men)with a median age of 63 years.The muscular manifestations included limb myalgia(100%),muscle weakness(57.14%)and rhabdomyolysis(14.29%).The most common extra-muscular manifestations were skin(42.86%)and kidney(33.33%)involvement.In 8 patients(47.06%,n=17),muscular involvement was the initial manifestation.The most frequent laboratory abnormalities included hyper-CK-emia(57.14%)and positive ANCA(52.38%).Necrotizing vasculitis was identified in 14 patients(66.67%)and multinucleated giant cells or granulomas in 4 patients(19.05%).Myofiber necrosis was observed in 8 patients(50%,n=16),neurogenic changes in 4 patients(25%,n=16)and no significant change in 4 patients.Corticosteroids(100%)and immunosuppressants/rituximab(85.71%)were the main agents prescribed.With a median follow-up of 12 months,sustained remissions were observed in17 patients(80.95%)and relapses occurred in 4 patients(19.05%).Conclusion:SM-MSVV should be considered in patients with limb myalgia,muscle weakness,fever,high CK level and extra-muscular involvements.The diagnosis of SM-MSVV should be applied when there was histologic evidence of vasculitis.Corticosteroid combined with immunosuppressant is effective for these patients.

关 键 词：骨骼肌血管炎 中-小血管炎 

分 类 号：R593.27[医药卫生—内科学]