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重叠性肌炎的特征和结局:来自 MyoCite 队列的成年人多群组队列比较研究。

Characteristics and outcomes of overlap myositis: a comparative multigroup cohort study in adults from the MyoCite cohort.

机构信息

Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, 226014, India.

出版信息

Rheumatol Int. 2021 Mar;41(3):551-563. doi: 10.1007/s00296-020-04779-y. Epub 2021 Jan 10.

Abstract

Overlap myositis (OM), an important subset of idiopathic inflammatory myopathies (IIM), is being increasingly recognized with wider myositis-specific autoantibody (MSA) testing. We studied the differences in clinical characteristics and long-term outcomes of OM with Dermatomyositis (DM), Polymyositis (PM), anti-synthetase syndrome (ASSD), and Cancer-associated IIM (CAM). Data from the MyoCite registry (Dec2017-May2020), a prospective dataset of IIM was extracted for the clinical profile, and MSAs, immunosuppressants received, disease activity (relapses and incomplete response), and treatment-related (drugs ADRs and infections) adverse events (DRAE and TRAE) were collected and analyzed between groups. Of 214 adults (58-OM,89-DM,27-ASSD,33-PM,7-CAM), OM had a greater female preponderance (13.5:1). Raynaud's and sclerodactyly were the prime distinguishing features of OM. OM could be distinguished from PM by frequent arthritis (OR-3.2) and infrequent dysphagia (OR-0.17); DM with greater nephritis (OR-20), infrequent dysphagia (OR-0.24) and rashes (OR-0.02); and ASSD by infrequent ILD (OR-0.07), and mechanic's hand (OR-0.05). 50% fulfilled the classification criteria for ASSD in the absence of MSA testing. ANA was positive more often (PM/DM: OR-6.7) and anti-Ro52 (OR-4.5) frequent in OM. Baseline serum creatinine and acute phase reactants were higher. OM received lower glucocorticoids (0 mg/kg, p < 0.001). Overall, 90% and 84% of OM at 12 and 24 months, respectively, achieved remission, with similar DRAE and TRAE as other IIM subsets. OM can be misdiagnosed as ASSD in the absence of MSA testing. Raynaud's, sclerodactyly, and a positive ANA may identify OM and prevent overtreatment.

摘要

重叠性肌炎(OM)是特发性炎症性肌病(IIM)的一个重要亚类,随着肌炎特异性自身抗体(MSA)检测范围的扩大,其越来越受到重视。我们研究了 OM 与皮肌炎(DM)、多发性肌炎(PM)、抗合成酶综合征(ASSD)和癌症相关的 IIM(CAM)在临床特征和长期结局方面的差异。从 MyoCite 登记处(2017 年 12 月至 2020 年 5 月)提取了 IIM 的前瞻性数据集,以提取临床特征和 MSA、接受的免疫抑制剂、疾病活动(复发和不完全缓解)以及与治疗相关的(药物不良反应和感染)不良事件(DRAE 和 TRAE),并对组间进行了分析。在 214 名成年人(58 例 OM、89 例 DM、27 例 ASSD、33 例 PM、7 例 CAM)中,OM 女性患者比例更高(13.5:1)。雷诺现象和硬皮病是 OM 的主要鉴别特征。OM 可通过频繁关节炎(OR-3.2)和不频繁吞咽困难(OR-0.17)与 PM 区分;DM 可通过更频繁肾炎(OR-20)、不频繁吞咽困难(OR-0.24)和皮疹(OR-0.02)与 OM 区分;ASSD 可通过不频繁间质性肺病(OR-0.07)和技工手(OR-0.05)与 OM 区分。在未进行 MSA 检测的情况下,有 50%的患者符合 ASSD 的分类标准。OM 中抗核抗体更常见(PM/DM:OR-6.7),抗 Ro52 抗体更常见(OR-4.5)。基线血清肌酐和急性期反应物较高。OM 接受的糖皮质激素剂量较低(0mg/kg,p<0.001)。OM 在 12 个月和 24 个月时分别有 90%和 84%达到缓解,与其他 IIM 亚组相比,DRAE 和 TRAE 相似。在未进行 MSA 检测的情况下,OM 可能被误诊为 ASSD。雷诺现象、硬皮病和抗核抗体阳性可能有助于识别 OM,并防止过度治疗。

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