{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,12]],"date-time":"2026-04-12T04:09:01Z","timestamp":1775966941705,"version":"3.50.1"},"reference-count":23,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2023,5,4]],"date-time":"2023-05-04T00:00:00Z","timestamp":1683158400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J of Rheum Dis"],"published-print":{"date-parts":[[2023,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Purpose<\/jats:title><jats:p>To conduct a systematic review with meta\u2010analysis to determine the effects of immunosuppression on Group 1 Pulmonary Arterial Hypertension in patients with systemic lupus erythematosus (SLE).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We searched Medline, Embase, Web of Science, <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/clinicaltrials.gov\">Clinicaltrials.gov<\/jats:ext-link>, and Cochrane Central Register of Controlled Trials (CENTRAL) with a search strategy developed by a medical librarian. We included retrospective, cross\u2010sectional, case\u2010control, prospective studies, and randomized controlled trials (RCTs) in our analysis and only included studies that contained data for patients with SLE. We included any immunosuppressive agents (including but not limited to cyclophosphamide, glucocorticoids, mycophenolate mofetil, azathioprine, and rituximab) We assessed for risk of bias and certainty of evidence. Outcomes included hemodynamics (as measured by pulmonary arterial hypertension), functional status, 6\u2009minute walk test (6MWT), quality of life, mortality, and serious adverse events.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We included three studies. One RCT and two single\u2010arm interventional observational studies. The RCT had a high risk of bias whereas the two single\u2010arm interventional studies were graded as fair quality. Meta\u2010analysis could not be conducted because of insufficient data. The RCT showed significant improvements in hemodynamics (as measured by pulmonary arterial pressures) and functional status. One observational study showed improvements in hemodynamics, functional status, and 6MWT. There were insufficient data for serious adverse events, mortality, and quality of life.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Despite a high prevalence and with a poor prognosis, there is a paucity of data for the role of immunosuppression in the treatment of Group 1 Pulmonary Arterial Hypertension in SLE. More high\u2010quality studies are needed, especially to investigate serious adverse events and quality of life.<\/jats:p><\/jats:sec>","DOI":"10.1111\/1756-185x.14706","type":"journal-article","created":{"date-parts":[[2023,5,4]],"date-time":"2023-05-04T13:00:06Z","timestamp":1683205206000},"page":"1022-1028","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Immunosuppression for the treatment of pulmonary hypertension in patients with systemic lupus erythematosus: A\u00a0systematic review"],"prefix":"10.1111","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3027-9287","authenticated-orcid":false,"given":"Sebastian","family":"Bruera","sequence":"first","affiliation":[{"name":"Section of Immunology, Allergy and Rheumatology Baylor College of Medicine  Houston Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arsha","family":"Sreedhar","sequence":"additional","affiliation":[{"name":"Department of Emergency Medicine McGovern Medical School  Houston Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sandeep K.","family":"Agarwal","sequence":"additional","affiliation":[{"name":"Section of Immunology, Allergy and Rheumatology Baylor College of Medicine  Houston Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Varsha","family":"Muthukumar","sequence":"additional","affiliation":[{"name":"Department of General Medicine Baylor College of Medicine  Houston Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yimin","family":"Geng","sequence":"additional","affiliation":[{"name":"Research Medical Library The University of Texas MD Anderson Cancer Center  Houston Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria A.","family":"Lopez\u2010Olivo","sequence":"additional","affiliation":[{"name":"Department of Health Services Research The University of Texas MD Anderson Cancer Center  Houston Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,5,4]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13075-019-1868-0"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00081-2018"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2015.11.012"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00008211"},{"key":"e_1_2_9_6_1","first-page":"1","article-title":"Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies","volume":"9","author":"Tselios K","year":"2017","journal-title":"Open Access Rheumatol"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.150451"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00296-012-2525-y"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1155\/2012\/854941"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2017.11.020"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1253\/circj.CJ-11-0473"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00003811"},{"key":"e_1_2_9_13_1","volume-title":"Cochrane Handbook for Systematic Reviews of Interventions version 6.3: Cochrane","author":"Higgins JPT","year":"2022"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.n71"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keab118"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1253\/circj.CJ-17-0351"},{"key":"e_1_2_9_17_1","article-title":"Endothelin receptor antagonists for pulmonary arterial hypertension","volume":"3","author":"Liu C","year":"2021","journal-title":"Cochrane Database Syst Rev"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.39489.470347.AD"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.l6890"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1191\/0961203304lu509oa"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kev097"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1177\/2045893217735820"},{"key":"e_1_2_9_23_1","unstructured":"XiaohongW.Clinical research on treatment of pulmonary arterial hypertension associated with connective tissue disease by Mycophenolate mofetil. International Clinical Trials Registry Platform ID \u2010 ChiCTR\u2010TRC\u2010140048942014."},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/1756-185X.14368"}],"container-title":["International Journal of Rheumatic Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/1756-185X.14706","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,20]],"date-time":"2023-08-20T03:31:52Z","timestamp":1692502312000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/1756-185X.14706"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5,4]]},"references-count":23,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2023,6]]}},"alternative-id":["10.1111\/1756-185X.14706"],"URL":"https:\/\/doi.org\/10.1111\/1756-185x.14706","archive":["Portico"],"relation":{},"ISSN":["1756-1841","1756-185X"],"issn-type":[{"value":"1756-1841","type":"print"},{"value":"1756-185X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,5,4]]},"assertion":[{"value":"2022-12-21","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-04-08","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-05-04","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}