The Prospective Registry Of MyositIS (PROMIS): II. Temporal shifts in causes of death among patients with idiopathic inflammatory myopathies

Chen Yu; Chanyuan Wu; Shangyi Jin; Liying Peng; Xinwang Duan; Pinting Yang; Wei Wei; Yanhong Wang; Mucong Li; Shuang Zhou; Shengyun Liu; Jing Wang; Hongbin Li; Zhaohui Zheng; Xiaofei Shi; Jian Xu; Yongfu Wang; Jiuliang Zhao; Dong Xu; Peng Yin; Xiaofeng Zeng; Mengtao Li; Qian Wang

doi:10.1016/j.jaad.2025.06.062

The Prospective Registry Of MyositIS (PROMIS): II. Temporal shifts in causes of death among patients with idiopathic inflammatory myopathies

J Am Acad Dermatol. 2025 Jul 11:S0190-9622(25)02399-0. doi: 10.1016/j.jaad.2025.06.062. Online ahead of print.

Authors

Chen Yu¹, Chanyuan Wu¹, Shangyi Jin¹, Liying Peng¹, Xinwang Duan², Pinting Yang³, Wei Wei⁴, Yanhong Wang⁵, Mucong Li¹, Shuang Zhou¹, Shengyun Liu⁶, Jing Wang⁷, Hongbin Li⁸, Zhaohui Zheng⁹, Xiaofei Shi¹⁰, Jian Xu¹¹, Yongfu Wang¹², Jiuliang Zhao¹, Dong Xu¹, Peng Yin¹³, Xiaofeng Zeng¹, Mengtao Li¹⁴, Qian Wang¹⁵

Affiliations

¹ Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science & Technology, Beijing, China.
² Department of Rheumatology, the Second Affiliated Hospital of Nanchang University, Nanchang, China.
³ Department of Rheumatology, the First Hospital of China Medical University, Shenyang, China.
⁴ Department of Rheumatology, Tianjin Medical University General Hospital, Tianjin, China.
⁵ Department of Epidemiology and Bio-statistics (YW), Institute of Basic Medical Science, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
⁶ Department of Rheumatology and Immunology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
⁷ Department of Rheumatology, the First People's Hospital of Yunnan Province, Kunming, China.
⁸ Department of Rheumatology, the Affiliated Hospital of Inner Mongolia Medical University, Hohhot, China.
⁹ Department of Clinical Immunology, Xijing Hospital, Fourth Military Medical University, Xi'an, China.
¹⁰ Department of Rheumatology, the First Affiliated Hospital of Henan University of Science & Technology, Luoyang, China.
¹¹ Department of Rheumatology, the First Affiliated Hospital of Kunming Medical University, Kunming, China.
¹² Department of Rheumatology, the First Affiliated Hospital of Baotou Medical College, Baotou, China.
¹³ National Center for Chronic and Noncommunicable Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Beijing, China. Electronic address: yinpeng@ncncd.chinacdc.cn.
¹⁴ Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science & Technology, Beijing, China. Electronic address: mengtao.li@cstar.org.cn.
¹⁵ Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science & Technology, Beijing, China; State Key Laboratory of Common Mechanism Research for Major Diseases, Beijing, China. Electronic address: wangqian_pumch@126.com.

PMID: 40653049
DOI: 10.1016/j.jaad.2025.06.062

Abstract

Background: Idiopathic inflammatory myopathies (IIM) are heterogeneous autoimmune diseases with variable long-term outcomes.

Objective: To determine the prognosis of IIM.

Methods: This multicenter, prospective cohort study included Chinese patients with IIM. We calculated the standardized mortality ratios (SMRs) adjusted for age and sex, analyzed causes of death, and identified risk factors for all-cause mortality using a multivariable Cox proportional hazards model.

Results: A total of 4,534 patients were included, with 2,902 dermatomyositis, 592 polymyositis, 927 anti-synthetase syndrome (ASS), and 113 immune-mediated necrotizing myositis (IMNM). 510 patients died with a median follow-up time of 46 months. The overall SMR was 6.2 (95% CI 5.7-6.7). Cumulative survival rates at 1, 3 and 10 years were 92.6%, 88.6% and 80.5%, respectively. Leading causes of death included interstitial lung diseases (18.8%), malignancies (18.8%), cardiovascular diseases (CVD, 18.8%), and infections (18.6%), which shifted over time (from ILD/infection to malignancies/CVD). Risk factors for mortality included male, lymphopenia, high serum ferritin, anti-MDA-5, anti-TIF1γ, and anti-Ro 52 antibodies.

Limitations: Underestimation of ASS and IMNM.

Conclusion: IIM patients had significantly higher mortality than general population, with variable causes of death and distinct risks based on myositis-specific antibodies. MSAs-based classification may provide deeper insights into pathogenesis and management of IIM.

Keywords: Idiopathic inflammatory myopathy; anti-synthetase syndrome; cause of death; dermatomyositis; immune-mediated necrotizing myopathy; mortality; myositis-specific autoantibody; polymyositis; prognosis; risk factor.