Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, 226014, India.
Rheumatol Int. 2021 Mar;41(3):551-563. doi: 10.1007/s00296-020-04779-y. Epub 2021 Jan 10.
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.
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。