{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,22]],"date-time":"2025-11-22T10:34:59Z","timestamp":1763807699951,"version":"3.37.3"},"reference-count":29,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2024,4,6]],"date-time":"2024-04-06T00:00:00Z","timestamp":1712361600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,10,15]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>To investigate the diagnostic and therapeutic landscape for patients with connective tissue disease (CTD) and CTD-associated pulmonary arterial hypertension (CTD-PAH) in acute-care general hospitals in Japan.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We conducted a retrospective cohort study by analysing the Medical Data Vision (MDV) database from April 2008 to September 2020. CTD patients who prescribed immunosuppressants were included in cohort 1, and CTD-PAH patients extracted from cohort 1 were included in cohort 2. Patient characteristics, diagnostic screening frequencies for PAH, and initial PAH-specific treatment patterns were assessed.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Overall, 16,648 patients with CTD and 81 patients with CTD-PAH were included in cohorts 1 and 2, respectively. The frequencies of screening tests for PAH, including brain natriuretic peptide (BNP), transthoracic echocardiogram (TTE), and \u2018diffusing capacity\u2019 of the \u2018lungs for carbon monoxide\u2019 (DLCO), among CTD patients were 0.7, 0.3, and 0.1 tests\/person-year, respectively. The most common initial PAH-specific treatment therapy was monotherapy (87.7%), followed by dual therapy (7.4%) and triple therapy (2.5%).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>This is the first study to describe the patient flow from PAH diagnosis to initial PAH-specific treatment for real-world patients who were followed regularly due to CTD in Japanese clinical practice.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/mr\/roae033","type":"journal-article","created":{"date-parts":[[2024,4,6]],"date-time":"2024-04-06T16:03:55Z","timestamp":1712419435000},"page":"1202-1212","source":"Crossref","is-referenced-by-count":3,"title":["Patient characteristics, diagnostic testing, and initial treatment profiles of patients with connective tissue disease-associated pulmonary arterial hypertension using a Japanese claims database"],"prefix":"10.1093","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-9556-5736","authenticated-orcid":false,"given":"Natsuko","family":"Tokushige","sequence":"first","affiliation":[{"name":"Medical Affairs Division, Janssen Pharmaceutical K.K. , Tokyo,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasuhiro","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Medical Affairs Division, Janssen Pharmaceutical K.K. , Tokyo,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Junichi","family":"Omura","sequence":"additional","affiliation":[{"name":"Medical Affairs Division, Janssen Pharmaceutical K.K. , Tokyo,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatsunori","family":"Jinnai","sequence":"additional","affiliation":[{"name":"Medical Affairs Division, Janssen Pharmaceutical K.K. , Tokyo,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatsuya","family":"Atsumi","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine, Hokkaido University , Sapporo,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2024,4,6]]},"reference":[{"key":"2024110410414142600_R1","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1253\/circj.CJ-66-0158","article-title":"Guidelines for the treatment of pulmonary hypertension (JCS 2017\/JPCPHS 2017)","volume":"83","author":"Fukuda","year":"2019","journal-title":"Circ J"},{"key":"2024110410414142600_R2","doi-asserted-by":"crossref","first-page":"932","DOI":"10.1016\/j.jpainsymman.2019.11.023","article-title":"Pulmonary arterial hypertension: a palliative medicine review of the disease, its therapies, and drug 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