Remission rates and predictors in idiopathic inflammatory myopathy subgroups: Insights from a single-center cohort.

Nantakarn Pongtarakulpanit,Sidra Tahir,Varshapriya Suresh,Vaidehi Kothari,Shiri Keret,Eugenia Gkiaouraki,Siamak Moghadam-Kia,Vladimir M Liarski,Dana P Ascherman,Chester V Oddis,Rohit Aggarwal
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Abstract

OBJECTIVE We evaluated remission rates and predictors across idiopathic inflammatory myopathy (IIM) subgroups using data from a prospective registry. METHODS Adult IIM patients with ≥ 1 year of disease duration, enrolled between 2000 and 2019, were analyzed. Subgroups included dermatomyositis (DM), antisynthetase syndrome (ASyS), and immune-mediated necrotizing myopathy (IMNM). Remission was defined as the absence of disease activity by expert assessment. Drug-free remission (DFR) and International Myositis Assessment and Clinical Studies Group (IMACS) remission (≥ 6 months of DFR) were also evaluated. Cumulative incidence of remission and flare was estimated using the Cumulative Incidence Function. Remission predictors were evaluated using cause-specific Cox proportional hazards models. The association between remission and mortality was assessed using Cox models with remission treated as a time-dependent covariate. RESULTS The cohort (n = 393) was 67.2% female with a mean age of 50.1 years. At 10 years, cumulative probabilities of remission, DFR, and IMACS remission were 40.3%, 23.3%, and 18.1%, respectively. Remission rates were highest in DM (47.4%) and lowest in ASyS (30.1%). Median time to first remission was 3.7 years. The 10-year cumulative incidence of flare following remission was 40.6%. Anti-Mi-2 antibody predicted a higher likelihood of remission (HR 2.08, p = 0.020). Remission and DFR were associated with improved survival. CONCLUSION Remission rates differed across IIM subgroups, being highest in DM and lowest in ASyS. Anti-Mi-2 antibody was associated with a higher likelihood of remission.
特发性炎性肌病亚组的缓解率和预测因素:来自单中心队列的见解。
目的:我们使用前瞻性登记数据评估特发性炎症性肌病(IIM)亚组的缓解率和预测因素。方法对2000年至2019年期间病程≥1年的成人IIM患者进行分析。亚组包括皮肌炎(DM)、抗合成酶综合征(ASyS)和免疫介导的坏死性肌病(IMNM)。专家评估的缓解定义为没有疾病活动。同时评估无药物缓解(DFR)和国际肌炎评估和临床研究组(IMACS)缓解(DFR≥6个月)。使用累积发生率函数估计缓解和发作的累积发生率。使用病因特异性Cox比例风险模型评估缓解预测因子。使用Cox模型评估缓解和死亡率之间的关系,缓解被视为时间相关协变量。结果393例患者中女性占67.2%,平均年龄50.1岁。10年后,累积缓解概率、DFR和IMACS缓解概率分别为40.3%、23.3%和18.1%。DM患者缓解率最高(47.4%),ASyS患者缓解率最低(30.1%)。首次缓解的中位时间为3.7年。缓解后10年累积发作率为40.6%。抗mi -2抗体预测更高的缓解可能性(HR 2.08, p = 0.020)。缓解和DFR与生存率的提高相关。结论IIM不同亚组的缓解率不同,DM患者缓解率最高,ASyS患者缓解率最低。抗mi -2抗体与更高的缓解可能性相关。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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