{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,15]],"date-time":"2025-08-15T00:45:55Z","timestamp":1755218755879,"version":"3.43.0"},"reference-count":20,"publisher":"PAGEPress Publications","issue":"1","license":[{"start":{"date-parts":[[2024,10,9]],"date-time":"2024-10-09T00:00:00Z","timestamp":1728432000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Reumatismo"],"abstract":"<jats:p>Objective. This study aimed to describe adult Brazilian and Japanese patients with anti-small ubiquitin-like modifier activating enzyme (SEA)-positive dermatomyositis (DM), as there are few studies in the literature. A literature review was also conducted.\nMethods. This bicentric international retrospective study, conducted between 2012 and 2023, included patients with anti-SAE-positive DM (2017 European League Against Rheumatism\/ American College of Rheumatology classification criteria). All demographic features and clinical, laboratory, therapeutic, and follow-up data were collected from Brazilian and Japanese centers using pre-standardized and parameterized information.\nResults. We included 17 adult patients with a median age of 65 years (56-76 years) and a predominance of females (82.4%). Constitutional symptoms at baseline were present in 58.8% of the patients. In addition to classical cutaneous DM lesions, one-third of the patients had myalgia and significant muscle weakness, whereas half presented with dysphagia, interstitial lung disease, and joint manifestations. The first-line treatment consisted of intravenous methylprednisolone and immunoglobulin pulse therapy in 41.2% and 28.6% of the patients, respectively. The median follow-up duration was 20 (13-74) months; at the last medical evaluation, half had active disease and were still using oral glucocorticoids (median dosage, 10.0 mg\/day). Approximately one-fifth to one-third of the patients were diagnosed with different types of cancer, had severe infections, or died.\nConclusions. Patients with anti-SAE-positive DM not only resemble the phenotype of antisynthetase syndrome but are also associated with a poor prognosis.<\/jats:p>","DOI":"10.4081\/reumatismo.2024.1686","type":"journal-article","created":{"date-parts":[[2024,10,9]],"date-time":"2024-10-09T07:28:18Z","timestamp":1728458898000},"source":"Crossref","is-referenced-by-count":1,"title":["Patients with anti-small ubiquitin-like modifier activating enzyme-positive dermatomyositis resembling antisynthetase syndrome with poor prognosis: a bicentric international retrospective study and literature review"],"prefix":"10.4081","volume":"77","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-5578-3143","authenticated-orcid":false,"given":"Camila Gusm\u00e3o Vicente","family":"De Carvalho","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Barbara","family":"Bayeh","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fernando Henrique Carlos","family":"De Souza","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2210-601X","authenticated-orcid":false,"given":"Renata","family":"Miossi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pleiades Tiharu","family":"Inaoka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takashi","family":"Matsushita","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naoki","family":"Mugii","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3682-4517","authenticated-orcid":false,"given":"Samuel Katsuyuki","family":"Shinjo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2549","published-online":{"date-parts":[[2024,10,9]]},"reference":[{"key":"9894","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2017-212786"},{"key":"9895","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2019.04.001"},{"key":"9896","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kead154"},{"key":"9897","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2014.08.011"},{"key":"9898","doi-asserted-by":"publisher","DOI":"10.3109\/08916934.2012.755958"},{"key":"9899","doi-asserted-by":"publisher","DOI":"10.1002\/acr2.11247"},{"key":"9900","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-017-00240-6"},{"key":"9901","doi-asserted-by":"publisher","DOI":"10.1002\/art.22862"},{"key":"9902","doi-asserted-by":"publisher","DOI":"10.1186\/s42358-022-00276-x"},{"key":"9903","doi-asserted-by":"publisher","DOI":"10.1016\/j.jim.2012.07.019"},{"key":"9904","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-019-04562-w"},{"key":"9905","doi-asserted-by":"publisher","DOI":"10.1155\/2022\/9000608"},{"key":"9906","doi-asserted-by":"publisher","DOI":"10.1186\/s12969-021-00532-2"},{"key":"9907","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2012-201736"},{"key":"9908","doi-asserted-by":"publisher","DOI":"10.1111\/1756-185X.13593"},{"key":"9909","doi-asserted-by":"publisher","DOI":"10.1007\/s12026-018-9006-7"},{"key":"9910","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2008.097162"},{"key":"9911","doi-asserted-by":"publisher","DOI":"10.1002\/art.21291"},{"key":"9912","doi-asserted-by":"publisher","DOI":"10.1067\/mjd.2003.199"},{"key":"9913","doi-asserted-by":"publisher","DOI":"10.6061\/clinics\/2013(07)04"}],"container-title":["Reumatismo"],"original-title":[],"link":[{"URL":"https:\/\/www.reumatismo.org\/reuma\/article\/download\/1686\/1050","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.reumatismo.org\/reuma\/article\/download\/1686\/1050","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,6]],"date-time":"2025-08-06T10:41:08Z","timestamp":1754476868000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.reumatismo.org\/reuma\/article\/view\/1686"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10,9]]},"references-count":20,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2025,2,13]]}},"URL":"https:\/\/doi.org\/10.4081\/reumatismo.2024.1686","relation":{},"ISSN":["2240-2683","0048-7449"],"issn-type":[{"type":"electronic","value":"2240-2683"},{"type":"print","value":"0048-7449"}],"subject":[],"published":{"date-parts":[[2024,10,9]]}}}