Long-term outcomes of idiopathic inflammatory myopathies: a large-scale longitudinal cohort study

IF 7.9 1区医学 Q1 IMMUNOLOGY

Journal of autoimmunity Pub Date : 2025-05-06 DOI:10.1016/j.jaut.2025.103435

Yiran Chen , Longyang Zhu , Chen Zong , Shiyu Wu , Xinxin Zhang , Lingling Huo , Yongpeng Ge , Xiaolan Tian , Fang Chen , Wei Jiang , Sizhao Li , Yu Zuo , Shanshan Li , Linrong He , Chunjia Li , Hanbo Yang , Xinyue Xiao , Lin Liang , Xia Liu , Lu Zhang , Qinglin Peng

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引用次数: 0

Abstract

Objectives

To investigate mortality, patient-reported outcomes (PROs), and drug-free remission (DFR) in a large well-characterised cohort of idiopathic inflammatory myopathies (IIMs).

Methods

This study retrospectively enrolled 1854 patients with IIMs. Follow-up lasted up to 20 years. Mortality was analysed using the standardised mortality ratio (SMR) and Kaplan-Meier survival analysis. PROs and DFR rates were examined in the survivors at the end of follow-up.

Results

Of 1854 patients, 348 (18.8 %) died during follow-up, with an overall SMR of 6.82 (95 % confidence interval [CI] 6.11–7.54). Subgroup analysis revealed the highest SMRs in dermatomyositis (DM), followed by antisynthetase syndrome (ASS), and immune-mediated necrotising myopathy, while SMRs in patients with polymyositis indicated no significant mortality difference from general population. Patients with anti-MDA5-positive DM exhibited higher SMRs than those with other IIM serotypes. Respiratory failure was the leading cause of death among patients with IIMs. Patients with DM had the lowest survival rates within the initial nine years of disease duration, whereas patients with ASS exhibited significantly reduced survival after nine years. At the end of follow-up, 17.1 % of patients achieved DFR (cumulative 3-, 5-, and 10-year DFR rates of 6.1 %, 14.9 %, and 29.3 %, respectively). Patients with DM presented with better PROs and higher DFR rates than those with other IIM subtypes.

Conclusions

Our data indicated increased mortality in patients with IIM compared with the general population and provided an important foundational understanding of IIMs. These findings emphasise the heterogeneity in the long-term outcomes across IIM subtypes, DM's acute nature, and ASS's progressive course.

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特发性炎性肌病的长期预后：一项大规模纵向队列研究

目的研究特发性炎症性肌病（IIMs）的死亡率、患者报告的预后（PROs）和无药缓解（DFR）。方法回顾性研究纳入1854例IIMs患者。随访时间长达20年。采用标准化死亡率（SMR）和Kaplan-Meier生存分析分析死亡率。在随访结束时检查幸存者的PROs和DFR率。结果1854例患者中，348例（18.8%）在随访期间死亡，总SMR为6.82（95%可信区间[CI] 6.11-7.54）。亚组分析显示，皮肌炎（DM）患者的smr最高，其次是抗合成酶综合征（ASS）和免疫介导的坏死性肌病，而多发性肌炎患者的smr与一般人群的死亡率无显著差异。抗mda5阳性DM患者的smr高于其他IIM血清型患者。呼吸衰竭是IIMs患者死亡的主要原因。糖尿病患者在最初9年内的生存率最低，而ASS患者在9年后的生存率显著降低。在随访结束时，17.1%的患者实现了DFR（累计3年、5年和10年DFR率分别为6.1%、14.9%和29.3%）。与其他IIM亚型相比，DM患者表现出更好的PROs和更高的DFR率。结论sour数据表明IIM患者的死亡率高于一般人群，为IIM提供了重要的基础认识。这些发现强调了IIM亚型、DM的急性性质和ASS的进展过程的长期结果的异质性。

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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来源期刊

Journal of autoimmunity 医学-免疫学

CiteScore

27.90

自引率

1.60%

发文量

117

审稿时长

17 days

期刊介绍： The Journal of Autoimmunity serves as the primary publication for research on various facets of autoimmunity. These include topics such as the mechanism of self-recognition, regulation of autoimmune responses, experimental autoimmune diseases, diagnostic tests for autoantibodies, as well as the epidemiology, pathophysiology, and treatment of autoimmune diseases. While the journal covers a wide range of subjects, it emphasizes papers exploring the genetic, molecular biology, and cellular aspects of the field. The Journal of Translational Autoimmunity, on the other hand, is a subsidiary journal of the Journal of Autoimmunity. It focuses specifically on translating scientific discoveries in autoimmunity into clinical applications and practical solutions. By highlighting research that bridges the gap between basic science and clinical practice, the Journal of Translational Autoimmunity aims to advance the understanding and treatment of autoimmune diseases.