{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T07:52:18Z","timestamp":1777881138794,"version":"3.51.4"},"reference-count":14,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2017,1,1]],"date-time":"2017-01-01T00:00:00Z","timestamp":1483228800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["SAGE Open Medical Case Reports"],"published-print":{"date-parts":[[2017,1,1]]},"abstract":"<jats:sec>\n                    <jats:title>Objective:<\/jats:title>\n                    <jats:p>The pulmonary vascular targeted treatment for systemic lupus erythematosus\u2013associated pulmonary arterial hypertension is similar to other connective tissue disease\u2013associated pulmonary arterial hypertension. In addition, there also appears to be a role for immunosuppression in the overall management. However, the optimal immunosuppressive regimen and what patients will respond to treatments are currently not clearly elucidated given the lack of randomized controlled trials on the subject. Our objective is to highlight the importance of early immunosuppression in systemic lupus erythematosus\u2013associated pulmonary arterial hypertension and the role of pulse dose steroids in management.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>\n                      This case describes a 23-year-old woman who presented with pulmonary arterial hypertension diagnosed by right heart catheterization with mean pulmonary artery pressure of 74 mmHg, pulmonary capillary wedge pressure of 12 mmHg, and a pulmonary vascular resistance of 1908 dyne s cm\n                      <jats:sup>\u22125<\/jats:sup>\n                      . Due to the aggressive nature of her disease, she declined despite management with epoprostenol and sildenafil. Because of coexisting systemic lupus erythematosus with hemolytic anemia and worsening pulmonary arterial hypertension, intensive immunosuppressive therapy with pulse dose steroids was initiated.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Shortly after initiation of pulse dose steroids and maintenance immunosuppression, she had a dramatic symptomatic and hemodynamic response with a decrease in her pulmonary vascular resistance from 1908 to 136 dyne sec cm\n                      <jats:sup>\u22125<\/jats:sup>\n                      and improvement in her mean pulmonary artery pressure from 74 to 27 mmHg on repeat right heart catheterization.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>Early immunosuppression is important to consider in those with systemic lupus erythematosus\u2013associated pulmonary arterial hypertension. Limited studies are available, but most have focused on the use of cyclophosphamide. Pulse dose steroids may be a potentially less toxic but equally effective manner to aid in the treatment of systemic lupus erythematosus\u2013pulmonary arterial hypertension when intensive immunosuppression is being considered.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/2050313x17707153","type":"journal-article","created":{"date-parts":[[2017,5,4]],"date-time":"2017-05-04T04:53:47Z","timestamp":1493873627000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":1,"title":["Pulse dose steroids in severe pulmonary arterial hypertension secondary to systemic lupus erythematosus"],"prefix":"10.1177","volume":"5","author":[{"given":"Cody","family":"Lee","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean","family":"Elwing","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care and Sleep Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2017,5,4]]},"reference":[{"key":"bibr1-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1155\/2012\/854941"},{"key":"bibr2-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200806-953OC"},{"key":"bibr3-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200510-1668OC"},{"key":"bibr4-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1177\/0961203314527366"},{"key":"bibr5-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2012.07.010"},{"key":"bibr6-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1002\/art.23303"},{"key":"bibr7-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1378\/chest.130.1.182"},{"key":"bibr8-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1191\/0961203304lu509oa"},{"key":"bibr9-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kev097"},{"key":"bibr10-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1177\/0961203311403347"},{"key":"bibr11-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1016\/0049-0172(84)90015-5"},{"key":"bibr12-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00003811"},{"key":"bibr13-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1177\/0961203314552461"},{"key":"bibr14-2050313X17707153","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00116313"}],"container-title":["SAGE Open Medical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/2050313X17707153","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/2050313X17707153","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/2050313X17707153","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:33:47Z","timestamp":1777595627000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/2050313X17707153"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,1,1]]},"references-count":14,"alternative-id":["10.1177\/2050313X17707153"],"URL":"https:\/\/doi.org\/10.1177\/2050313x17707153","relation":{},"ISSN":["2050-313X","2050-313X"],"issn-type":[{"value":"2050-313X","type":"print"},{"value":"2050-313X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,1,1]]},"article-number":"2050313X17707153"}}