Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes Rares d'Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM U1016, Institut Cochin, Paris; Université Paris Descartes, Sorbonne Paris Cité, Paris; CNRS UMR 8104, Paris.
Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes Rares d'Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France.
Autoimmun Rev. 2020 Jan;19(1):102431. doi: 10.1016/j.autrev.2019.102431. Epub 2019 Nov 14.
Little is known about systemic sclerosis (SSc)-associated myopathy (SScAM) treatment. Herein we evaluated the use of intravenous immunoglobulin (IVIg) in SScAM.
We conducted a retrospective study of patients with SScAM in the Internal medicine department of Cochin University Hospital between 1993 and 2017.
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.
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.
系统性硬化症（SSc）相关肌病（SScAM）的治疗方法知之甚少。在此，我们评估了静脉注射免疫球蛋白（IVIg）在 SScAM 中的应用。
我们对 1993 年至 2017 年期间在 Cochin 大学医院内科就诊的 SScAM 患者进行了回顾性研究。
共纳入 52 例患者，其中 18 例（34.6%）为局限型 SSc，34 例（65.4%）为弥漫型 SSc。SScAM 发生在 SSc 诊断后中位数[四分位间距（IQR）]时间为 1 个月[0-15]。34 例（65.4%）患者有肌无力，28 例（53.8%）有肌痛，24 例（46.2%）有吞咽困难。50 例（96.2%）患者肌酸激酶升高，22/26（84.6%）例肌电图呈肌病表现，10/12（83.3%）例 MRI 显示肩带肌肉高信号，49/50（98%）例肌肉活检异常。18 例（34.6%）患者接受 IVIg 治疗。18 例患者中有 3 例（16.7%）发生严重不良事件。与未接受 IVIg 治疗的患者相比，接受 IVIg 治疗的患者接受最大剂量皮质类固醇（CS）的时间显著延长，治疗 3 个月时 CS 减少更明显，1 年和随访结束时 CS 剂量更低。
本研究表明 IVIg 作为辅助治疗具有良好的耐受性，支持其作为 CS 节约剂用于 SScAM。
