{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T23:28:54Z","timestamp":1787009334407,"version":"build-2736575974"},"reference-count":20,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2022,2,3]],"date-time":"2022-02-03T00:00:00Z","timestamp":1643846400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,6,24]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>A 25-year-old woman was admitted to our hospital with severe pulmonary arterial hypertension associated with systemic lupus erythematosus (SLE-PAH). Her mean pulmonary arterial pressure was 56\u2009mmHg, and her SLE Disease Activity Index-2\u2009K score was 14 on admission. In addition to a strong immunosuppressive regimen, which included steroid pulse therapy followed by high-dose oral prednisolone (1\u2009mg\/kg) and intravenous cyclophosphamide, an upfront combination of vasodilator therapy, including oral tadalafil, macitentan, and intravenous epoprostenol, was administered in the early phase. Two months later, her mean pulmonary arterial pressure was 29\u2009mmHg, and her other haemodynamic markers showed significant improvement. She refused to start life-long intravenous epoprostenol therapy and so was switched to oral selexipag and inhaled iloprost. The transition was successful, and she has experienced no exacerbations of SLE-PAH during the 10\u2009months since the onset of pulmonary arterial hypertension. To the best of our knowledge, this is the first report of intravenous epoprostenol being switched to alternative oral and inhaled therapy in a patient with SLE-PAH. In combination with adequate immunosuppressive therapy, it is probably easier to make this transition in patients with SLE-PAH than in those with pulmonary arterial hypertension of a different aetiology. Continuous infusion of epoprostenol can have potentially life-threatening complications and a detrimental effect on the quality of life. Our alternative treatment strategy was successful, and we hope that it will prove beneficial in other cases.<\/jats:p>","DOI":"10.1093\/mrcr\/rxac009","type":"journal-article","created":{"date-parts":[[2022,2,8]],"date-time":"2022-02-08T15:37:42Z","timestamp":1644334662000},"page":"183-188","source":"Crossref","is-referenced-by-count":4,"title":["Successful transition from intravenous epoprostenol to oral selexipag and inhaled iloprost in a case of severe pulmonary arterial hypertension associated with systemic lupus erythematosus"],"prefix":"10.1093","volume":"6","author":[{"given":"Chihiro","family":"Saiki","sequence":"first","affiliation":[{"name":"Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka , Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0344-0242","authenticated-orcid":false,"given":"Yusuke","family":"Kashiwado","sequence":"additional","affiliation":[{"name":"Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka , Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taku","family":"Yokoyama","sequence":"additional","affiliation":[{"name":"Department of Hematology, Oncology and Cardiovascular Medicine, Kyushu University Hospital , Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahiro","family":"Ayano","sequence":"additional","affiliation":[{"name":"Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka , Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keisuke","family":"Imabayashi","sequence":"additional","affiliation":[{"name":"Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka , Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shotaro","family":"Kawano","sequence":"additional","affiliation":[{"name":"Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka , Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuhiko","family":"Higashioka","sequence":"additional","affiliation":[{"name":"Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka , Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasutaka","family":"Kimoto","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Kyushu University Beppu Hospital , Beppu, Oita, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mitsuhiro","family":"Fukata","sequence":"additional","affiliation":[{"name":"Department of Hematology, Oncology and Cardiovascular Medicine, Kyushu University Hospital , Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroki","family":"Mitoma","sequence":"additional","affiliation":[{"name":"Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka , Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nobuyuki","family":"Ono","sequence":"additional","affiliation":[{"name":"Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka , Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yojiro","family":"Arinobu","sequence":"additional","affiliation":[{"name":"Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka , Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koichi","family":"Akashi","sequence":"additional","affiliation":[{"name":"Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka , Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takahiko","family":"Horiuchi","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Kyushu University Beppu Hospital , Beppu, Oita, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroaki","family":"Niiro","sequence":"additional","affiliation":[{"name":"Department of Medical Education, Graduate School of Medical Sciences, Kyushu University , Fukuoka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,2,3]]},"reference":[{"key":"2022062410205590900_R1","doi-asserted-by":"crossref","first-page":"1519","DOI":"10.1007\/s10067-013-2307-2","article-title":"Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review of the literature","volume":"32","author":"Yang","year":"2013","journal-title":"Clin Rheumatol"},{"key":"2022062410205590900_R2","doi-asserted-by":"crossref","first-page":"1431","DOI":"10.1016\/j.rmed.2008.04.016","article-title":"Evaluation of disease-specific health-related quality of life in patients with pulmonary arterial hypertension","volume":"102","author":"Zlupko","year":"2008","journal-title":"Respir Med"},{"key":"2022062410205590900_R3","article-title":"Prostacyclin for pulmonary arterial hypertension","volume":"5","author":"Barnes","year":"2019","journal-title":"Cochrane Database Syst Rev"},{"key":"2022062410205590900_R4","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1056\/NEJM199602013340504","article-title":"A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension","volume":"334","author":"Barst","year":"1996","journal-title":"N Engl J Med"},{"key":"2022062410205590900_R5","doi-asserted-by":"crossref","first-page":"409","DOI":"10.7326\/0003-4819-121-6-199409150-00003","article-title":"Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin","volume":"121","author":"Barst","year":"1994","journal-title":"Ann Intern Med"},{"key":"2022062410205590900_R6","doi-asserted-by":"crossref","first-page":"2522","DOI":"10.1056\/NEJMoa1503184","article-title":"Selexipag for the treatment of pulmonary arterial hypertension","volume":"373","author":"Sitbon","year":"2015","journal-title":"N Engl J Med"},{"key":"2022062410205590900_R7","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1007\/s40256-017-0262-z","article-title":"Targeting the prostacyclin pathway with selexipag in patients with pulmonary arterial hypertension receiving double combination therapy: insights from the randomized controlled GRIPHON study","volume":"18","author":"Coghlan","year":"2018","journal-title":"Am J Cardiovasc Drugs"},{"key":"2022062410205590900_R8","doi-asserted-by":"crossref","first-page":"381","DOI":"10.4103\/2045-8932.114768","article-title":"A multicenter, retrospective study of patients with pulmonary arterial hypertension transitioned from parenteral prostacyclin therapy to inhaled iloprost","volume":"3","author":"Channick","year":"2013","journal-title":"Pulm Circ"},{"key":"2022062410205590900_R9","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1080\/17476348.2017.1317599","article-title":"The prostacyclin pathway in pulmonary arterial hypertension: a clinical review","volume":"11","author":"Pozo","year":"2017","journal-title":"Expert Rev Respir Med"},{"key":"2022062410205590900_R10","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1378\/chest.130.1.182","article-title":"Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension","volume":"130","author":"Sanchez","year":"2006","journal-title":"Chest"},{"key":"2022062410205590900_R11","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1002\/art.23303","article-title":"Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases","volume":"58","author":"Jais","year":"2008","journal-title":"Arthritis Rheum"},{"key":"2022062410205590900_R12","doi-asserted-by":"crossref","first-page":"2668","DOI":"10.1253\/circj.CJ-11-0473","article-title":"Intensive immunosuppressive therapy improves pulmonary arterial hypertension associated with connective tissue disease","volume":"75","author":"Miyamichi-Yamamoto","year":"2011","journal-title":"Circ J"},{"key":"2022062410205590900_R13","doi-asserted-by":"crossref","first-page":"1673","DOI":"10.1093\/rheumatology\/kev097","article-title":"Pulmonary arterial hypertension in systemic lupus erythematosus may benefit by addition of immunosuppression to vasodilator therapy: an observational study","volume":"54","author":"Kommireddy","year":"2015","journal-title":"Rheumatology (Oxford)"},{"key":"2022062410205590900_R14","doi-asserted-by":"crossref","first-page":"546","DOI":"10.1253\/circj.CJ-17-0351","article-title":"Predictors of favorable responses to immunosuppressive treatment in pulmonary arterial hypertension associated with connective tissue disease","volume":"82","author":"Yasuoka","year":"2018","journal-title":"Circ J"},{"key":"2022062410205590900_R15","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1093\/eurheartj\/ehv317","article-title":"2015 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"37","author":"Gali\u00e8","year":"2016","journal-title":"Eur Heart J"},{"key":"2022062410205590900_R16","doi-asserted-by":"crossref","DOI":"10.1183\/16000617.0055-2016","article-title":"Epoprostenol and pulmonary arterial hypertension: 20 years of clinical experience","volume":"26","author":"Sitbon","year":"2017","journal-title":"Eur Respir Rev"},{"key":"2022062410205590900_R17","doi-asserted-by":"crossref","first-page":"425","DOI":"10.7326\/0003-4819-132-6-200003210-00002","article-title":"Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease: a randomized, controlled trial","volume":"132","author":"Badesch","year":"2000","journal-title":"Ann Intern Med"},{"key":"2022062410205590900_R18","doi-asserted-by":"crossref","first-page":"1211","DOI":"10.3109\/s10165-012-0828-1","article-title":"Intravenous epoprostenol treatment of patients with connective tissue disease and pulmonary arterial hypertension at a single center","volume":"23","author":"Shirai","year":"2013","journal-title":"Mod Rheumatol"},{"key":"2022062410205590900_R19","doi-asserted-by":"crossref","first-page":"2119","DOI":"10.1016\/S0735-1097(03)00463-7","article-title":"Beraprost therapy for pulmonary arterial hypertension","volume":"41","author":"Barst","year":"2003","journal-title":"J Am Coll Cardiol"},{"key":"2022062410205590900_R20","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1016\/j.jjcc.2015.11.012","article-title":"Rapid and high-dose titration of epoprostenol improves pulmonary hemodynamics and clinical outcomes in patients with idiopathic and heritable pulmonary arterial hypertension","volume":"68","author":"Tokunaga","year":"2016","journal-title":"J Cardiol"}],"container-title":["Modern Rheumatology Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/mrcr\/advance-article-pdf\/doi\/10.1093\/mrcr\/rxac009\/42786595\/rxac009.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/mrcr\/article-pdf\/6\/2\/183\/44245174\/rxac009.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/mrcr\/article-pdf\/6\/2\/183\/44245174\/rxac009.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,6,24]],"date-time":"2022-06-24T06:21:33Z","timestamp":1656051693000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/mrcr\/article\/6\/2\/183\/6520713"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,2,3]]},"references-count":20,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2022,2,3]]},"published-print":{"date-parts":[[2022,6,24]]}},"URL":"https:\/\/doi.org\/10.1093\/mrcr\/rxac009","relation":{},"ISSN":["2472-5625"],"issn-type":[{"value":"2472-5625","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2022,6,1]]},"published":{"date-parts":[[2022,2,3]]}}}