{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T03:47:33Z","timestamp":1792640853476,"version":"4.5.0"},"reference-count":30,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,3,17]],"date-time":"2023-03-17T00:00:00Z","timestamp":1679011200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81971532"],"award-info":[{"award-number":["81971532"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81971533"],"award-info":[{"award-number":["81971533"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>There is substantial heterogeneity among the phenotypes of patients with anti\u2013melanoma differentiation\u2013associated gene 5 antibody\u2013positive (anti\u2010MDA5+) dermatomyositis (DM), hindering disease assessment and management. This study aimed to identify distinct phenotype groups in patients with anti\u2010MDA5+ DM and to determine the utility of these phenotypes in predicting patient outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A total of 265 patients with anti\u2010MDA5+ DM were retrospectively enrolled in the study. An unsupervised hierarchical cluster analysis was performed to characterize the different phenotypes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients were stratified into 3 clusters characterized by markedly different features and outcomes. Cluster 1 (n\u00a0=\u00a0108 patients) was characterized by mild risk of rapidly progressive interstitial lung disease (RPILD), with the cumulative incidence of non\u2010RPILD being 85.2%. Cluster 2 (n\u00a0=\u00a072 patients) was characterized by moderate risk of RPILD, with the cumulative incidence of non\u2010RPILPD being 73.6%. Patients in cluster 3 (n\u00a0=\u00a085 patients), which was characterized by a high risk of RPILD and a cumulative non\u2010RPILD incidence of 32.9%, were more likely than patients in the other 2 subgroups to have anti\u2013Ro 52 antibodies in conjunction with high titers of anti\u2010MDA5 antibodies. All\u2010cause mortality rates of 60%, 9.7%, and 3.7% were determined for clusters 3, 2, and 1, respectively (<jats:italic>P<\/jats:italic> &lt;\u20090.0001). Decision tree analysis led to the development of a simple algorithm for anti\u2010MDA5+ DM patient classification that included the following 8 variables: age &gt;50\u2009years, disease course of &lt;3 months, myasthenia (proximal muscle weakness), arthritis, C\u2010reactive protein level, creatine kinase level, anti\u2013Ro 52 antibody titer, and anti\u2010MDA5 antibody titer. This algorithm placed patients in the appropriate cluster with 78.5% accuracy in the development cohort and 70.0% accuracy in the external validation cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Cluster analysis identified 3 distinct clinical patterns and outcomes in our large cohort of anti\u2010MDA5+ DM patients. Classification of DM patients into phenotype subgroups with prognostic values may help physicians improve the efficacy of clinical decision\u2010making.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.42308","type":"journal-article","created":{"date-parts":[[2022,7,18]],"date-time":"2022-07-18T16:14:11Z","timestamp":1658160851000},"page":"609-619","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":87,"title":["Identification of Three Different Phenotypes in Anti\u2013Melanoma Differentiation\u2013Associated Gene 5 Antibody\u2013Positive Dermatomyositis Patients: Implications for Prediction of Rapidly Progressive Interstitial Lung Disease"],"prefix":"10.1002","volume":"75","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3418-0727","authenticated-orcid":false,"given":"Lingxiao","family":"Xu","sequence":"first","affiliation":[{"name":"Department of Rheumatology The First Affiliated Hospital of Nanjing Medical University  Nanjing 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China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7943-1572","authenticated-orcid":false,"given":"Fenghong","family":"Yuan","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology Wuxi People's Hospital  Wuxi China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ju","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology Huai'an First People's Hospital  Huai'an Jiangsu China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology The First People's Hospital of Changzhou  Changzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhanyun","family":"Da","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology Affiliated Hospital of Nantong University  Nantong 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