Coexistence of Anti-Ro52 Antibodies in Anti-MDA5 Antibody-Positive Dermatomyositis Is Highly Associated With Rapidly Progressive Interstitial Lung Disease and Mortality Risk

Chengyin Lv; Hanxiao You; Lingxiao Xu; Lei Wang; Fenghong Yuan; Ju Li; Min Wu; Shiliang Zhou; Zhanyun Da; Jie Qian; Hua Wei; Wei Yan; Lei Zhou; Yan Wang; Songlou Yin; Dongmei Zhou; Jian Wu; Yan Lu; Dinglei Su; Zhichun Liu; Lin Liu; Longxin Ma; Xiaoyan Xu; Yinshan Zang; Huijie Liu; Tianli Ren; Fang Wang; Miaojia Zhang; Wenfeng Tan

doi:10.3899/jrheum.220139

Coexistence of Anti-Ro52 Antibodies in Anti-MDA5 Antibody-Positive Dermatomyositis Is Highly Associated With Rapidly Progressive Interstitial Lung Disease and Mortality Risk

J Rheumatol. 2023 Feb;50(2):219-226. doi: 10.3899/jrheum.220139. Epub 2022 Jun 15.

Authors

Chengyin Lv¹, Hanxiao You¹, Lingxiao Xu¹, Lei Wang¹, Fenghong Yuan², Ju Li³, Min Wu⁴, Shiliang Zhou⁴, Zhanyun Da⁵, Jie Qian⁵, Hua Wei⁶, Wei Yan⁶, Lei Zhou⁷, Yan Wang⁷, Songlou Yin⁸, Dongmei Zhou⁸, Jian Wu⁹, Yan Lu¹⁰, Dinglei Su¹¹, Zhichun Liu¹², Lin Liu¹³, Longxin Ma¹⁴, Xiaoyan Xu¹⁵, Yinshan Zang¹⁶, Huijie Liu¹⁷, Tianli Ren¹⁸, Fang Wang¹⁹, Miaojia Zhang²⁰, Wenfeng Tan²⁰

Affiliations

¹ C. Lv, MD, H. You, MD, PhD, L. Xu, MD, PhD, L. Wang, MD, PhD, M. Zhang, MD, W. Tan, MD, PhD, Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing.
² F. Yuan, MD, Department of Rheumatology and Immunology, The Affiliated Wuxi People's Hospital of Nanjing Medical University, Wuxi.
³ J. Li, MD, Department of Rheumatology, Huai'an First People's Hospital, Huai'an, Jiangsu.
⁴ M. Wu, MD, S. Zhou, MD, Department of Rheumatology, The First People's Hospital of Changzhou, Changzhou.
⁵ Z. Da, MD, J. Qian, MD, Department of Rheumatology, Affiliated Hospital of Nantong University, Nantong.
⁶ H. Wei, MD, W. Yan, MD, Department of Rheumatology, Northern Jiangsu People's Hospital, Yangzhou.
⁷ L. Zhou, MD, Y. Wang, MD, Department of Rheumatology, Changzhou No.2 People's Hospital, Changzhou.
⁸ S. Yin, MD, D. Zhou, MD, Department of Rheumatology, The Affiliated Hospital of Xuzhou Medical University, Xuzhou.
⁹ J. Wu, MD, Department of Rheumatology, The First Affiliated Hospital of Soochow University, Suzhou.
¹⁰ Y. Lu, MD, Department of Rheumatology, Jiangsu Province Hospital of Chinese Medicine, Nanjing.
¹¹ D. Su, MD, Department of Rheumatology, Nanjing First Hospital, Nanjing.
¹² Z. Liu, MD, Department of Rheumatology, The Second Affiliated Hospital of Soochow University, Suzhou.
¹³ L. Liu, MD, Department of Rheumatology, Xuzhou Central Hospital, Xuzhou.
¹⁴ L. Ma, MD, Department of Rheumatology, Yancheng No.1 People's Hospital, Yancheng.
¹⁵ X. Xu, MD, Department of Rheumatology, Zhongda Hospital Southeast University, Nanjing.
¹⁶ Y. Zang, MD, Department of Rheumatology, The Affiliated Suqian First People's Hospital of Nanjing Medical University, Suqian.
¹⁷ H. Liu, MD, Department of Rheumatology, The First People's Hospital of Lianyungang, Lianyungang.
¹⁸ T. Ren, MD, Department of Rheumatology, Wuxi No.2 People's Hospital, Wuxi.
¹⁹ F. Wang, MD, PhD, Department of Cardiology, The First Affiliated Hospital of Nanjing Medical University, China, Nanjing, Jiangsu, China.
²⁰ C. Lv, MD, H. You, MD, PhD, L. Xu, MD, PhD, L. Wang, MD, PhD, M. Zhang, MD, W. Tan, MD, PhD, Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing; [email protected] [email protected].

PMID: 35705235
DOI: 10.3899/jrheum.220139

Abstract

Objective: Interstitial lung disease (ILD) is a common extramuscular complication contributing to significant morbidity and mortality in patients with dermatomyositis (DM) who are positive for antimelanoma differentiation-associated gene 5 antibody (anti-MDA5+). We conducted this study to investigate the association of anti-Ro52 antibodies with clinical characteristics and prognosis in patients with anti-MDA5+ DM.

Methods: We assessed a cohort of 246 patients with anti-MDA5+ DM. To calculate hazard ratios and 95% CIs for rapidly progressive ILD (RP-ILD) and death while controlling for potential confounders, variables selected by univariate Cox regression analysis were included in a multivariate Cox regression model with the stepwise forward-selection method. A 2-tailed analysis with P < 0.05 was considered to be statistically significant.

Results: A total of 246 patients with anti-MDA5+ DM were enrolled; 70 patients were male, and the patient group had an average age of 53.1 (12.4) years. Anti-Ro52 was present in 64.2% (158/246) patients. Patients with anti-MDA5+ DM who were positive for anti-Ro52 had a higher rate of RP-ILD (log-rank P < 0.001) and a higher mortality rate (log-rank P = 0.01). For patients with anti-MDA5+ DM who were positive for anti-Ro52, those with a short disease course and high inflammation were at increased risk of RP-ILD and death. The appearance of active rash was an independent protective factor of death.

Conclusion: Anti-Ro52 antibodies were highly prevalent in patients with anti-MDA5+ DM, and their coexistence correlated with a higher rate of RP-ILD and mortality. Patients with a short disease course, with increased inflammation, and without rash were more likely to have a poor prognosis.

Keywords: MDA5; RP-ILD; anti-Ro52; dermatomyositis; interstitial lung disease.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Autoantibodies
Dermatomyositis* / complications
Disease Progression
Female
Humans
Inflammation / complications
Interferon-Induced Helicase, IFIH1
Lung Diseases, Interstitial* / etiology
Male
Middle Aged
Prognosis
Retrospective Studies

Substances

Autoantibodies
Interferon-Induced Helicase, IFIH1