{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,2]],"date-time":"2026-11-02T17:56:49Z","timestamp":1793642209679,"version":"4.5.1"},"reference-count":30,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,1,10]],"date-time":"2024-01-10T00:00:00Z","timestamp":1704844800000},"content-version":"vor","delay-in-days":9,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Immunity Inflam &amp;amp; Disease"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Little is known about the features of macrophage activation syndrome (MAS) in dermatomyositis, especially the association between rapidly progressive interstitial lung disease (RP\u2010ILD) and MAS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To determine the characteristics of MAS in patients with dermatomyositis and their association with RP\u2010ILD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This was a retrospective cohort study of 201 dermatomyositis patients at the First Affiliated Hospital of Zhejiang University over a 10\u2010year period.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 22 (10.9%) patients were diagnosed with MAS. The rate of RP\u2010ILD was significantly higher in patients with MAS than in those without MAS (81.8% vs. 17.4%, respectively, <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001). Multivariate analysis indicated that RP\u2010ILD (<jats:italic>p<\/jats:italic>\u2009=\u2009.019), ferritin level\u2009&gt;\u20091685\u2009ng\/mL (<jats:italic>p<\/jats:italic>\u2009=\u2009.007) and hemoglobin\u2009&lt;\u2009100\u2009g\/L (<jats:italic>p<\/jats:italic>\u2009=\u2009.001) were independent risk factors for MAS. Furthermore, RP\u2010ILD patients with MAS presented more cardiac injury (50.0% vs. 13.3%, respectively, <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.009), central nervous system dysfunction (42.8% vs. 3.4%, respectively, <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001) and hemorrhage (38.9% vs. 3.3%, respectively, <jats:italic>p<\/jats:italic>\u2009=\u2009.003) than RP\u2010ILD patients without MAS. The 90\u2010day cumulative survival rate for patients with MAS was significantly lower than for those without MAS (18.2% vs. 82.1%, respectively, <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>MAS was a common and fatal complication of dermatomyositis in our cohort. MAS is closely related to RP\u2010ILD in patients with dermatomyositis. When RP\u2010ILD is present in dermatomyositis patients with abnormal laboratory findings, such as cytopenia and hyperferritinemia, the presence of MAS should be considered.<\/jats:p><\/jats:sec>","DOI":"10.1002\/iid3.1141","type":"journal-article","created":{"date-parts":[[2024,1,10]],"date-time":"2024-01-10T14:14:31Z","timestamp":1704896071000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Clinical features of macrophage activation syndrome in adult dermatomyositis:\u00a0A single\u2010center retrospective case\u2010control study"],"prefix":"10.1002","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0216-0644","authenticated-orcid":false,"given":"Dingxian","family":"Zhu","sequence":"first","affiliation":[{"name":"Department of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine  Hangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuni","family":"Ying","sequence":"additional","affiliation":[{"name":"Department of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine  Hangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Changyi","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine  Hangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4641-7907","authenticated-orcid":false,"given":"Sheng","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine  Hangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shunli","family":"Tang","sequence":"additional","affiliation":[{"name":"Department of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine  Hangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chuanyin","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, The First Affiliated Hospital Zhejiang University School of Medicine  Hangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4940-9662","authenticated-orcid":false,"given":"Hong","family":"Fang","sequence":"additional","affiliation":[{"name":"Department of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine  Hangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3386-297X","authenticated-orcid":false,"given":"Jianjun","family":"Qiao","sequence":"additional","affiliation":[{"name":"Department of Dermatology, The First Affiliated Hospital Zhejiang University School of Medicine  Hangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,1,10]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-015-8496-5"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-007-0562-9"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keaa034"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keac627"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/cei.13404"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.3928\/19382359-20170717-01"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2014.07.007"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2020.12.007"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keq073"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0b013e31824227b9"},{"key":"e_1_2_9_12_1","unstructured":"BohanA PeterJ. 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