{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,6]],"date-time":"2025-05-06T09:12:44Z","timestamp":1746522764973},"reference-count":30,"publisher":"Oxford University Press (OUP)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,7,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>Real-world evidence regarding prevalence, patient characteristics, and treatment patterns for pulmonary arterial hypertension (PAH) related to systemic lupus erythematosus (SLE) in Japan is limited.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We conducted a retrospective study analysing Japan\u2019s Medical Data Vision database from April 2008 to September 2020. Prevalence, incidence, patient characteristics, treatment patterns, and use of vasodilators by treatment line were evaluated.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>The prevalence of PAH was 0.392% in SLE patients (n\u2009=\u2009114\/29,077). Cumulative incidence was 0.53% (3\u2009years) and 0.77% (5\u2009years). Of 114 SLE\u2013PAH patients, 49% developed PAH &amp;lt;1\u2009year from SLE diagnosis. SLE\u2013PAH patients were predominantly females (88% vs. 72%), had a lower mean age at SLE diagnosis (53 vs. 56\u2009years), and had more severe SLE (61% vs. 25%) than non-PAH\u2013SLE patients. Glucocorticoids (58%) and vasodilators (27%) were preferred first-line monotherapy for SLE\u2013PAH. A combination of glucocorticoids and immunosuppressants (19%) was the predominant first-line combination therapy. Endothelin receptor antagonists (40% and 44%) and nitric oxide analogues (31% and 40%) were dominant first- and second-line vasodilators.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>SLE\u2013PAH patients were predominantly females, were younger at diagnosis, and had more severe SLE than non-PAH\u2013SLE patients. Most were diagnosed &amp;lt;1\u2009year of SLE diagnosis. In Japan\u2019s real-world practice, the initial treatment goal is SLE management, while vasodilators are preferred in advanced diseases, as per the Medical Data Vision database.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/mr\/road090","type":"journal-article","created":{"date-parts":[[2023,9,25]],"date-time":"2023-09-25T15:49:55Z","timestamp":1695656995000},"page":"741-749","source":"Crossref","is-referenced-by-count":3,"title":["Prevalence, patient characteristics and\u00a0treatment patterns among systemic lupus erythematosus\u2013pulmonary arterial hypertension patients in\u00a0real-world clinical practice: A retrospective analysis of\u00a0Medical Data Vision Database in\u00a0Japan"],"prefix":"10.1093","volume":"34","author":[{"given":"Tatsuya","family":"Atsumi","sequence":"first","affiliation":[{"name":"Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Hokkaido, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jin Yu","family":"Tan","sequence":"additional","affiliation":[{"name":"Janssen Pharmaceutical Asia Pacific , Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ashley Pei-Chun","family":"Chiang","sequence":"additional","affiliation":[{"name":"Janssen Pharmaceutical Asia Pacific , Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dae Young","family":"Yu","sequence":"additional","affiliation":[{"name":"Janssen Pharmaceutical Asia Pacific , Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yogeshwar","family":"Makanji","sequence":"additional","affiliation":[{"name":"Janssen Pharmaceutical Asia Pacific , Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David Bin-Chia","family":"Wu","sequence":"additional","affiliation":[{"name":"Janssen Pharmaceutical Asia Pacific , Singapore"},{"name":"Saw Swee Hock School of Public Health, National University of Singapore , Singapore, Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeremy","family":"Casorso","sequence":"additional","affiliation":[{"name":"Prospection Pty Ltd , Sydney, NSW, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mahsa","family":"Kouhkamari","sequence":"additional","affiliation":[{"name":"Prospection Pty Ltd , Sydney, NSW, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sooyeol","family":"Lim","sequence":"additional","affiliation":[{"name":"Prospection KK , Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2023,9,25]]},"reference":[{"key":"2024070612132719000_R1","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1016\/j.ccm.2010.05.001","article-title":"Pulmonary manifestations of\u00a0systemic lupus erythematosus","volume":"31","author":"Kamen","year":"2010","journal-title":"Clin 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