特发性炎症性肌病患者死亡原因的前瞻性登记研究（PROMIS）：II. 特发性炎症性肌病患者死亡原因的时间变化

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

作者信息

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

机构信息

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

Department of Rheumatology, The Second Affiliated Hospital of Nanchang University, Nanchang, China.

出版信息

J Am Acad Dermatol. 2025 Jul 11. doi: 10.1016/j.jaad.2025.06.062.

DOI:10.1016/j.jaad.2025.06.062

PMID:40653049

Abstract

BACKGROUND

Idiopathic inflammatory myopathies (IIMs) 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 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 4534 patients were included, with 2902 dermatomyositis, 592 polymyositis, 927 anti-synthetase syndrome, and 113 immune-mediated necrotizing myopathy. Five hundred ten patients died with a median follow-up time of 46 months. The overall standardized mortality ratio 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 (18.8%), and infections (18.6%), which shifted over time (from interstitial lung disease/infection to malignancies/cardiovascular diseases). Risk factors for mortality included male, lymphopenia, high serum ferritin, anti-melanoma differentiation-associated gene 5, anti-transcription intermediary factor 1 gamma (anti-TIF1γ), and anti-Ro 52 antibodies.

LIMITATIONS

Underestimation of anti-synthetase syndrome and immune-mediated necrotizing myositis.

CONCLUSION

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

摘要

背景

特发性炎性肌病（IIM）是一类具有异质性的自身免疫性疾病，长期预后各不相同。

目的

确定IIM的预后情况。

方法

这项多中心前瞻性队列研究纳入了中国的IIM患者。我们计算了经年龄和性别调整的标准化死亡比，分析了死亡原因，并使用多变量Cox比例风险模型确定全因死亡的危险因素。

结果

共纳入4534例患者，其中皮肌炎2902例、多发性肌炎592例、抗合成酶综合征927例、免疫介导的坏死性肌病113例。510例患者死亡，中位随访时间为46个月。总体标准化死亡比为6.2（95%CI：5.7 - 6.7）。1年、3年和10年的累积生存率分别为92.6%、88.6%和80.5%。主要死亡原因包括间质性肺疾病（18.8%）、恶性肿瘤（18.8%）、心血管疾病（18.8%）和感染（18.6%），且这些死因随时间推移有所变化（从间质性肺疾病/感染转变为恶性肿瘤/心血管疾病）。死亡的危险因素包括男性、淋巴细胞减少、高血清铁蛋白、抗黑色素瘤分化相关基因5、抗转录中介因子1γ（抗TIF1γ）和抗Ro 52抗体。