Time-dependent changes in RPILD and mortality risk in anti-MDA5+ DM patients: a cohort study of 272 cases in China,Rheumatology

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Time-dependent changes in RPILD and mortality risk in anti-MDA5+ DM patients: a cohort study of 272 cases in China
Rheumatology ( IF 4.7 ) Pub Date : 2022-08-12 , DOI: 10.1093/rheumatology/keac450
Hanxiao You ₁ , Lei Wang ₁ , Jiajia Wang ₁ , Chengyin Lv ₁ , Lingxiao Xu ₁ , Fenghong Yuan ₂ , Ju Li ₃ , Min Wu ₄ , Shiliang Zhou ₄ , Zhanyun Da ₅ , Jie Qian ₅ , Hua Wei ₆ , Wei Yan ₆ , Lei Zhou ₇ , Yan Wang ₇ , Songlou Yin ₈ , Dongmei Zhou ₈ , Jian Wu ₉ , Yan Lu ₁₀ , Dinglei Su ₁₁ , Zhichun Liu ₁₂ , Lin Liu ₁₃ , Longxin Ma ₁₄ , Xiaoyan Xu ₁₅ , Yinshan Zang ₁₆ , Huijie Liu ₁₇ , Tianli Ren ₁₈ , Fang Wang ₁₉ , Miaojia Zhang ₁ , Wenfeng Tan ₁

Affiliation

Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing.
Department of Rheumatology and Immunology, the Affiliated Wuxi People's Hospital of Nanjing Medical University, Wuxi.
Department of Rheumatology, Huai'an First People's Hospital, Huai'an.
Department of Rheumatology, The First People's Hospital of Changzhou, Changzhou.
Department of Rheumatology, Affiliated Hospital of Nantong University, Nantong.
Department of Rheumatology, Northern Jiangsu People's Hospital, Yangzhou.
Department of Rheumatology, Changzhou No.2 People's Hospital, Changzhou.
Department of Rheumatology, The Affiliated Hospital of Xuzhou Medical University, Xuzhou.
Department of Rheumatology, The First Affiliated Hospital of Soochow University, Suzhou.
Department of Rheumatology, Jiangsu Province Hospital of Chinese Medicine.
Department of Rheumatology, Nanjing First Hospital, Nanjing.
Department of Rheumatology, The Second Affiliated Hospital of Soochow University, Suzhou.
Department of Rheumatology, Xuzhou Central Hospital, Xuzhou.
Department of Rheumatology, Yancheng No.1 People's Hospital, Yancheng.
Department of Rheumatology, Zhongda Hospital Southeast University, Nanjing.
Department of Rheumatology, The Affiliated Suqian First People's Hospital of Nanjing Medical University, Suqian.
Department of Rheumatology, The First People's Hospital of Lianyungang, Lianyungang.
Department of Rheumatology, Wuxi No.2 People's Hospital, Wuxi.
Department of Cardiology, The First Affiliated Hospital of Nanjing Medical University, China, Nanjing, Jiangsu, China.

Objectives Anti-melanoma differentiation-associated gene 5 positive (anti-MDA5+) dermatomyositis (DM) has a close relationship with rapidly progressive interstitial lung disease (RPILD) and is associated with high mortality. However, data regarding the time-dependent risk of RPILD and deaths during disease progression are limited. We conducted this study to investigate whether the risk of RPILD and death were time-dependent or not in anti-MDA5+ DM. Methods We assessed a cohort of 272 patients with anti-MDA5+ DM. The clinical characteristics of patients with anti-MDA5+ were collected, and COX regression was used to analyze independent risk factors for RPILD and death. We also described changes in risk of RPILD and death over time and their potential clinical implications. Results There were 272 anti-MDA5+ DM patients enrolled in this study. According to the multivariate cox regression analysis, short disease course, high CRP level, anti-Ro52 positive, and anti-MDA5 titer ( ++∼ +++) were independent risk factors of RPILD. High CK level, high CRP level, and RPILD were independent risk factors for death. >90% RPILD and 84% mortality occurred in the first 6 months after disease onset. Notably, the first 3-months is a particularly high-risk period, with 50% RPILD and 46% death occurring. Hazards regarding RPILD and mortality diminished over time during a median follow-up of 12 months. Conclusion These results suggest significant, time-dependent changes in RPILD and mortality risk in anti-MDA5+ DM patients, providing a cutoff time window to estimate disease progression and poor prognosis.

更新日期：2022-08-12

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