Identification of Three Different Phenotypes in Anti–Melanoma Differentiation–Associated Gene 5 Antibody–Positive Dermatomyositis Patients: Implications for Prediction of Rapidly Progressive Interstitial Lung Disease

• Published in: Arthritis & rheumatology (Hoboken, N.J.) N.J.), 2023-04, Vol.75 (4), p.609-619
• Main Authors: Xu, Lingxiao, You, Hanxiao, Wang, Lei, Lv, Chengyin, Yuan, Fenghong, Li, Ju, Wu, Min, Da, Zhanyun, Wei, Hua, Yan, Wei, Zhou, Lei, Yin, Songlou, Zhou, Dongmei, Wu, Jian, Lu, Yan, Su, Dinglei, Liu, Zhichun, Liu, Lin, Ma, Longxin, Xu, Xiaoyan, Zang, Yinshan, Liu, Huijie, Ren, Tianli, Wang, Fang, Du, Yan, Xue, Jing, Zhang, Miaojia, Tan, Wenfeng
• Format: Article
• Language: English
• Subjects: Algorithms; Antibodies; Arthritis; Autoantibodies; Classification; Cluster analysis; Creatine; Creatine kinase; Decision analysis; Decision making; Decision trees; Dermatomyositis; Differentiation; Disease Progression; Heterogeneity; Humans; Interferon-Induced Helicase, IFIH1; Kinases; Lung diseases; Lung Diseases, Interstitial; Melanoma; Myasthenia; Neuromuscular junctions; Patients; Phenotype; Phenotypes; Prognosis; Retrospective Studies; Risk; Subgroups
• ISSN: 2326-5191; 2326-5205
• DOI: 10.1002/art.42308

Objective There is substantial heterogeneity among the phenotypes of patients with anti–melanoma differentiation–associated gene 5 antibody–positive (anti‐MDA5+) dermatomyositis (DM), hindering disease assessment and management. This study aimed to identify distinct phenotype groups in patients with anti‐MDA5+ DM and to determine the utility of these phenotypes in predicting patient outcomes. Methods A total of 265 patients with anti‐MDA5+ DM were retrospectively enrolled in the study. An unsupervised hierarchical cluster analysis was performed to characterize the different phenotypes. Results Patients were stratified into 3 clusters characterized by markedly different features and outcomes. Cluster 1 (n = 108 patients) was characterized by mild risk of rapidly progressive interstitial lung disease (RPILD), with the cumulative incidence of non‐RPILD being 85.2%. Cluster 2 (n = 72 patients) was characterized by moderate risk of RPILD, with the cumulative incidence of non‐RPILPD being 73.6%. Patients in cluster 3 (n = 85 patients), which was characterized by a high risk of RPILD and a cumulative non‐RPILD incidence of 32.9%, were more likely than patients in the other 2 subgroups to have anti–Ro 52 antibodies in conjunction with high titers of anti‐MDA5 antibodies. All‐cause mortality rates of 60%, 9.7%, and 3.7% were determined for clusters 3, 2, and 1, respectively (P 50 years, disease course of