{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T01:03:27Z","timestamp":1792026207112,"version":"4.4.0"},"reference-count":35,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,2,2]],"date-time":"2023-02-02T00:00:00Z","timestamp":1675296000000},"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":["81971531"],"award-info":[{"award-number":["81971531"]}],"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":["82171788"],"award-info":[{"award-number":["82171788"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Intern Med"],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To explore the role of peripheral lymphocyte count in phenotyping and prognosis prediction in dermatomyositis (DM) patients with anti\u2010MDA5 antibodies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In total, 1669 patients with idiopathic inflammatory myopathy (IIM) were retrospectively enrolled. Clinical characteristics and prognosis of patients with anti\u2010MDA5+ DM were analyzed in association with peripheral lymphocyte counts and clusters determined by unsupervised machine learning.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The peripheral lymphocyte count was significantly lower in the anti\u2010MDA5+ DM group (<jats:italic>N<\/jats:italic>\u00a0=\u00a0421) than in the other IIM serotype groups. The anti\u2010MDA5+ DM patients were divided into three groups; the severe lymphopenia group had skin ulcers and rapidly progressive interstitial lung disease (RP\u2010ILD); patients with a normal lymphocyte count had a younger age of onset, more frequent arthritis, and normal serum ferritin levels, whereas mild lymphopenia group showed a moderate increase of serum ferritin and intermediate incidence of RP\u2010ILD. Survival analysis revealed that the 3\u2010 and 6\u2010month mortality rates were significantly higher in the severe lymphopenia group (29.0% and 42.1%, respectively) than in the mild lymphopenia group and normal lymphocyte count group (<jats:italic>p<\/jats:italic> value &lt;0.001). Consistently, unsupervised machine learning identified three similar groups; the arthritis cluster shows the highest lymphocyte counts and best prognosis; the RP\u2010ILD cluster presents the lowest peripheral lymphocyte, high incidence of RP\u2010ILD, and poor prognosis; the typical DM rash cluster had a moderate peripheral lymphocyte count and an intermediate prognosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Lymphopenia is a unique manifestation of anti\u2010MDA5+ DM. Peripheral lymphocyte count can define clinical phenotypes and predict prognosis in anti\u2010MDA5+ DM.<\/jats:p><\/jats:sec>","DOI":"10.1111\/joim.13607","type":"journal-article","created":{"date-parts":[[2023,1,22]],"date-time":"2023-01-22T15:24:46Z","timestamp":1674401086000},"page":"494-507","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":68,"title":["Peripheral lymphocyte count defines the clinical phenotypes and prognosis in patients with anti\u2010MDA5\u2010positive dermatomyositis"],"prefix":"10.1111","volume":"293","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3821-0698","authenticated-orcid":false,"given":"Qiwen","family":"Jin","sequence":"first","affiliation":[{"name":"Peking University China\u2010Japan Friendship School of Clinical Medicine  Beijing China"},{"name":"Department of Rheumatology Key Lab of Myositis China\u2010Japan Friendship Hospital  Beijing 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