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

作者：Lingxiao Xu, Hanxiao You, Lei Wang, Chengyin Lv, Fenghong Yuan, Ju Li, Min Wu, Zhanyun Da, Hua Wei, Wei Yan, Lei Zhou, Songlou Yin, Dongmei Zhou, Jian Wu, Yan Lü, Dinglei Su, Zhichun Liu, Lin Liu, Longxin Ma, Xiaoyan Xu, Yinshan Zang, Huijie Liu, Tianli Ren, Fang Wang, Yan Du, Jing Xue, Miaojia Zhang, Wenfeng Tan · 发表于：Arthritis & Rheumatology · 年份：2022 · DOI：10.1002/art.42308 · 被引用次数：95 · 研究领域：Inflammatory Myopathies and Dermatomyositis、Systemic Sclerosis and Related Diseases、Parkinson's Disease and Spinal Disorders

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 < 0.0001). Decision tree analysis led to the development of a simple algorithm for anti-MDA5+ DM patient c...