INTRODUCTION Little is known about systemic sclerosis (SSc)-associated myopathy (SScAM) treatment. Herein we evaluated the use of intravenous immunoglobulin (IVIg) in SScAM. METHODS We conducted a retrospective study of patients with SScAM in the Internal medicine department of Cochin University Hospital between 1993 and 2017. RESULTS Fifty-two patients were included comprising 18 (34.6%) with limited SSc and 34 (65.4%) with diffuse SSc. SScAM occurred at a median [interquartile range (IQR)] time of 1 month [0-15] after SSc diagnosis. Thirty-four patients (65.4%) had muscle weakness, 28 (53.8%) had myalgia and 24 (46.2%) had dysphagia. Fifty patients (96.2%) had increased creatine kinase, 22/26 (84.6%) had myopathic electromyography, 10/12 (83.3%) had a high intensity signal of girdle muscles on MRI and 49/50 (98%) had abnormal muscle biopsy. Eighteen (34.6%) patients received IVIg. Severe adverse events occurred in 3/18 (16.7%) patients. When compared to patients who did not receive IVIg, patients who received IVIg had a significantly higher maximal corticosteroid (CS) dose ever, a greater decrease of CS at 3 months, and a lower CS dose at one year and at the end of follow up. CONCLUSIONS This study suggests the benefit of IVIg as adjunctive therapy, with an acceptable tolerance profile, and supports its use as a CS-sparing agent, in SScAM.

中文翻译：

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静脉注射免疫球蛋白在全身性硬化症相关性肌病中节省皮质类固醇的益处：一项在52例患者中进行的比较研究。

引言关于全身性硬化症（SSc）相关性肌病（SScAM）的治疗知之甚少。本文中，我们评估了静脉注射免疫球蛋白（IVIg）在SScAM中的使用。方法我们回顾性研究了1993年至2017年间在Cochin大学医院内科的SScAM患者。结果52例患者包括18例（34.6％）有限SSc和34例（65.4％）弥漫性SSc。诊断SScAM发生在SSc诊断后1个月[0-15]的中位[四分位间距（IQR）]时间。三十四名患者（65.4％）出现肌无力，28例（53.8％）患有肌痛，24例（46.2％）出现吞咽困难。五十名患者（96.2％）的肌酸激酶升高，22/26（84.6％）的肌病性肌电图，10/12（83.3％）的患者在MRI上显示腰带肌强度高信号和49/50（98％）的患者肌肉异常活检。十八名（34.6％）患者接受了IVIg。3/18（16.7％）患者发生了严重的不良事件。与未接受IVIg的患者相比，接受IVIg的患者有史以来最大皮质类固醇（CS）剂量明显更高，3个月时CS下降幅度更大，一年及随访结束时CS剂量更低。结论这项研究表明IVIg作为辅助治疗的益处，具有可接受的耐受性，并支持其在SScAM中用作CS保留剂。