{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T09:44:57Z","timestamp":1790675097314,"version":"4.1.0"},"reference-count":7,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2026,1,19]],"date-time":"2026-01-19T00:00:00Z","timestamp":1768780800000},"content-version":"vor","delay-in-days":2314,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2019,9,19]],"date-time":"2019-09-19T00:00:00Z","timestamp":1568851200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2019,12,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Optimal management of systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) remains unclear. Our observation describes the case of a 31-year-old SLE patient presenting with cardiogenic shock revealing severe PAH, in which a therapeutic scheme combining immunosuppressants (pulse cyclophosphamide and corticosteroids) and PAH-specific drugs (bosentan, tadalafil, and epoprostenol) led to a complete normalization of pulmonary haemodynamics and allowed a progressive weaning of PAH vasodilators. This case report supports the efficacy of immunosuppressants and use of PAH-specific therapy as a bridge therapy in severe SLE-PAH. Further studies on larger population are required to confirm these findings.<\/jats:p>","DOI":"10.1002\/ehf2.12507","type":"journal-article","created":{"date-parts":[[2019,9,19]],"date-time":"2019-09-19T16:14:33Z","timestamp":1568909673000},"page":"1322-1325","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Efficacy of Immunosuppressants with Bridge Vasodilator Therapy in Severe\n                    <i>Lupus Erythematosus<\/i>\n                    -Associated Pulmonary Arterial Hypertension"],"prefix":"10.1093","volume":"6","author":[{"given":"S\u00e9bastien","family":"Sanges","sequence":"first","affiliation":[{"name":"UFR M\u00e9decine, Univ. Lille , F-59000, Lille ,","place":["France"]},{"name":"INSERM, U995, LIRIC - Lille Inflammation Research International Center , Lille ,","place":["France"]},{"name":"D\u00e9partement de M\u00e9decine Interne et Immunologie Clinique, CHU Lille , Lille ,","place":["France"]},{"name":"Centre National de R\u00e9f\u00e9rence Maladies Syst\u00e9miques et Auto-immnues Rares (Scl\u00e9rodermie Syst\u00e9mique) , Lille ,","place":["France"]},{"name":"Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNECT , Lille ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurent","family":"Savale","sequence":"additional","affiliation":[{"name":"Univ. Paris-Sud, Facult\u00e9 de M\u00e9decine, Universit\u00e9 Paris-Saclay , Le Kremlin-Bic\u00eatre, Paris ,","place":["France"]},{"name":"AP-HP, Service de Pneumologie, DHU Thorax Innovation, H\u00f4pital Bic\u00eatre , Le Kremlin-Bic\u00eatre, Paris ,","place":["France"]},{"name":"INSERM U999, Centre Chirurgical Marie-Lannelongue, LabEx LERMIT , Le Plessis-Robinson ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Lamblin","sequence":"additional","affiliation":[{"name":"Univ. Lille, CHU Lille, Service de Cardiologie, Institut Pasteur , Lille ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martine","family":"R\u00e9my-Jardin","sequence":"additional","affiliation":[{"name":"CHU Lille, Service d'Imagerie Thoracique , Lille ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Humbert","sequence":"additional","affiliation":[{"name":"Univ. Paris-Sud, Facult\u00e9 de M\u00e9decine, Universit\u00e9 Paris-Saclay , Le Kremlin-Bic\u00eatre, Paris ,","place":["France"]},{"name":"AP-HP, Service de Pneumologie, DHU Thorax Innovation, H\u00f4pital Bic\u00eatre , Le Kremlin-Bic\u00eatre, Paris ,","place":["France"]},{"name":"INSERM U999, Centre Chirurgical Marie-Lannelongue, LabEx LERMIT , Le Plessis-Robinson ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Sobanski","sequence":"additional","affiliation":[{"name":"UFR M\u00e9decine, Univ. Lille , F-59000, Lille ,","place":["France"]},{"name":"INSERM, U995, LIRIC - Lille Inflammation Research International Center , Lille ,","place":["France"]},{"name":"D\u00e9partement de M\u00e9decine Interne et Immunologie Clinique, CHU Lille , Lille ,","place":["France"]},{"name":"Centre National de R\u00e9f\u00e9rence Maladies Syst\u00e9miques et Auto-immnues Rares (Scl\u00e9rodermie Syst\u00e9mique) , Lille ,","place":["France"]},{"name":"Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNECT , Lille ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2019,9,19]]},"reference":[{"key":"2026012115084802800_ehf212507-bib-0001","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.01887-2018","article-title":"Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives","volume":"53","author":"Humbert","year":"2019","journal-title":"Eur Respir J"},{"key":"2026012115084802800_ehf212507-bib-0002","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.01913-2018","article-title":"Haemodynamic definitions and updated clinical classification of pulmonary hypertension","volume":"53","author":"Simonneau","year":"2019","journal-title":"Eur Respir J"},{"key":"2026012115084802800_ehf212507-bib-0003","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/j.chest.2017.08.014","article-title":"French Collaborators Recruiting Members(*). Pulmonary arterial hypertension associated with systemic lupus erythematosus: results from the French Pulmonary Hypertension Registry","volume":"153","author":"Hachulla","year":"2018","journal-title":"Chest"},{"key":"2026012115084802800_ehf212507-bib-0004","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":"2026012115084802800_ehf212507-bib-0005","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 & Rheumatism"},{"key":"2026012115084802800_ehf212507-bib-0006","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1007\/s11926-015-0560-x","article-title":"Current approaches to the treatment of systemic-sclerosis-associated pulmonary arterial hypertension (SSc-PAH)","volume":"18","author":"Sobanski","year":"2016","journal-title":"Curr Rheumatol Rep"},{"key":"2026012115084802800_ehf212507-bib-0007","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":"Circulation Journal"}],"container-title":["ESC Heart Failure"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fehf2.12507","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ehf2.12507","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ehf2.12507","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/academic.oup.com\/eschf\/article-pdf\/6\/6\/1322\/66135899\/eschf_6_6_1322.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/eschf\/article-pdf\/6\/6\/1322\/66135899\/eschf_6_6_1322.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,21]],"date-time":"2026-01-21T20:11:00Z","timestamp":1769026260000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/eschf\/article\/6\/6\/1322\/8403503"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,9,19]]},"references-count":7,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2019,9,19]]},"published-print":{"date-parts":[[2019,12,1]]}},"URL":"https:\/\/doi.org\/10.1002\/ehf2.12507","relation":{},"ISSN":["2055-5822"],"issn-type":[{"value":"2055-5822","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2019,12]]},"published":{"date-parts":[[2019,9,19]]},"assertion":[{"value":"2019-05-11","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-07-17","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-09-19","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}