{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T13:33:25Z","timestamp":1777901605682,"version":"3.51.4"},"reference-count":66,"publisher":"Springer Science and Business Media LLC","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,11,26]]},"DOI":"10.7759\/cureus.97863","type":"journal-article","created":{"date-parts":[[2025,11,26]],"date-time":"2025-11-26T15:55:31Z","timestamp":1764172531000},"source":"Crossref","is-referenced-by-count":1,"title":["Pulmonary Hypertension in Systemic Lupus Erythematosus: Current Evidence and Future Directions"],"prefix":"10.7759","author":[{"given":"Juan Camilo","family":"Santacruz","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marta Juliana","family":"Mantilla","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sandra","family":"Pulido","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carlos","family":"Agudelo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Juli\u00e1n Alberto","family":"Naranjo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oscar Vicente","family":"Vergara","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John","family":"Londono","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2010.135186","article-title":"Therapeutic opportunities in systemic lupus erythematosus: state of the art and prospects for the new decade","volume":"69","author":"Bertsias GK","year":"2010","unstructured":"Bertsias GK, Salmon JE, Boumpas DT. Therapeutic opportunities in systemic lupus erythematosus: state of the art and prospects for the new decade. Ann Rheum Dis. 2010, 69:1603-11. 10.1136\/ard.2010.135186","journal-title":"Ann Rheum Dis"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1136\/thorax.55.2.159","article-title":"Pleuropulmonary manifestations of systemic lupus erythematosus","volume":"55","author":"Keane MP","year":"2000","unstructured":"Keane MP, Lynch JP 3rd. Pleuropulmonary manifestations of systemic lupus erythematosus. Thorax. 2000, 55:159-66. 10.1136\/thorax.55.2.159","journal-title":"Thorax"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-022-06446-y","article-title":"Pulmonary hypertension in connective tissue diseases: epidemiology, pathogenesis, and treatment","volume":"42","author":"Cansu D\u00dc","year":"2023","unstructured":"Cansu D\u00dc, Korkmaz C. Pulmonary hypertension in connective tissue diseases: epidemiology, pathogenesis, and treatment. Clin Rheumatol. 2023, 42:2601-10. 10.1007\/s10067-022-06446-y","journal-title":"Clin Rheumatol"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1155\/2012\/854941","article-title":"Pulmonary arterial hypertension in systemic lupus erythematosus: current status and future direction","volume":"2012","author":"Dhala A","year":"2012","unstructured":"Dhala A. Pulmonary arterial hypertension in systemic lupus erythematosus: current status and future direction. Clin Dev Immunol. 2012, 2012:854941. 10.1155\/2012\/854941","journal-title":"Clin Dev Immunol"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.2174\/1573397113666170704102444","article-title":"Cardiovascular and pulmonary manifestations of systemic lupus erythematosus","volume":"13","author":"Tselios K","year":"2017","unstructured":"Tselios K, Urowitz MB. Cardiovascular and pulmonary manifestations of systemic lupus erythematosus. Curr Rheumatol Rev. 2017, 13:206-18. 10.2174\/1573397113666170704102444","journal-title":"Curr Rheumatol Rev"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00182813","article-title":"Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients","volume":"44","author":"Hao YJ","year":"2014","unstructured":"Hao YJ, Jiang X, Zhou W, et al.. Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients. Eur Respir J. 2014, 44:963-72. 10.1183\/09031936.00182813","journal-title":"Eur Respir J"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kep203","article-title":"Prevalence and risk factors for pulmonary arterial hypertension in patients with lupus","volume":"48","author":"Prabu A","year":"2009","unstructured":"Prabu A, Patel K, Yee CS, et al.. Prevalence and risk factors for pulmonary arterial hypertension in patients with lupus. Rheumatology (Oxford). 2009, 48:1506-11. 10.1093\/rheumatology\/kep203","journal-title":"Rheumatology (Oxford)"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1002\/pul2.70145","article-title":"Characterization of the population with intermediate-risk status in pulmonary arterial hypertension: patients in a Latin American country: the CAPRI Registry","volume":"15","author":"Conde-Camacho R","year":"2025","unstructured":"Conde-Camacho R, Orozco-Levi M, Londo\u00f1o A, et al.. Characterization of the population with intermediate-risk status in pulmonary arterial hypertension: patients in a Latin American country: the CAPRI Registry. Pulm Circ. 2025, 15:e70145. 10.1002\/pul2.70145","journal-title":"Pulm Circ"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehv317","article-title":"2015 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT)","volume":"37","author":"Gali\u00e8 N","year":"2016","unstructured":"Gali\u00e8 N, Humbert M, Vachiery JL, et al.. 2015 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016, 37:67-119. 10.1093\/eurheartj\/ehv317","journal-title":"Eur Heart J"},{"key":"ref10","article-title":"Pulmonary hypertension in systemic lupus erythematosus: clinical association and survival in 18 patients","volume":"26","author":"Li EK","year":"1999","unstructured":"Li EK, Tam LS. Pulmonary hypertension in systemic lupus erythematosus: clinical association and survival in 18 patients. J Rheumatol. 1999, 26:1923-9.","journal-title":"J Rheumatol"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2023.103506","article-title":"Pulmonary arterial hypertension associated with connective tissue diseases (CTD-PAH): recent and advanced data","volume":"23","author":"Thoreau B","year":"2024","unstructured":"Thoreau B, Mouthon L. Pulmonary arterial hypertension associated with connective tissue diseases (CTD-PAH): recent and advanced data. Autoimmun Rev. 2024, 23:103506. 10.1016\/j.autrev.2023.103506","journal-title":"Autoimmun Rev"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1002\/art.30541","article-title":"Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival","volume":"63","author":"Humbert M","year":"2011","unstructured":"Humbert M, Yaici A, de Groote P, et al.. Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum. 2011, 63:3522-30. 10.1002\/art.30541","journal-title":"Arthritis Rheum"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01913-2018","article-title":"Haemodynamic definitions and updated clinical classification of pulmonary hypertension","volume":"53","author":"Simonneau G","year":"2019","unstructured":"Simonneau G, Montani D, Celermajer DS, et al.. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019, 53:1801913. 10.1183\/13993003.01913-2018","journal-title":"Eur Respir J"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.3390\/ijms24065085","article-title":"Systemic lupus erythematosus and pulmonary hypertension","volume":"24","author":"Parperis K","year":"2023","unstructured":"Parperis K, Velidakis N, Khattab E, Gkougkoudi E, Kadoglou NP. Systemic lupus erythematosus and pulmonary hypertension. Int J Mol Sci. 2023, 24:5085. 10.3390\/ijms24065085","journal-title":"Int J Mol Sci"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1111\/1756-185X.13478","article-title":"Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR-PAH study: baseline characteristics and risk factors","volume":"22","author":"Zhang N","year":"2019","unstructured":"Zhang N, Li M, Qian J, et al.. Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR-PAH study: baseline characteristics and risk factors. Int J Rheum Dis. 2019, 22:921-8. 10.1111\/1756-185X.13478","journal-title":"Int J Rheum Dis"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200510-1668OC","article-title":"Pulmonary arterial hypertension in France: results from a national registry","volume":"173","author":"Humbert M","year":"2006","unstructured":"Humbert M, Sitbon O, Chaouat A, et al.. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006, 173:1023-30. 10.1164\/rccm.200510-1668OC","journal-title":"Am J Respir Crit Care Med"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00057010","article-title":"Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension","volume":"36","author":"Humbert M","year":"2010","unstructured":"Humbert M, Sitbon O, Ya\u00efci A, et al.. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J. 2010, 36:549-55. 10.1183\/09031936.00057010","journal-title":"Eur Respir J"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951121000688","article-title":"Risk factors of pulmonary arterial hypertension in patients with systemic lupus erythematosus","volume":"31","author":"Lei Y","year":"2021","unstructured":"Lei Y, Zhang X, Feng Y, Wang J, Luo R. Risk factors of pulmonary arterial hypertension in patients with systemic lupus erythematosus. Cardiol Young. 2021, 31:1619-24. 10.1017\/S1047951121000688","journal-title":"Cardiol Young"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1007\/s00296-011-1880-4","article-title":"Clinical features and independent predictors of pulmonary arterial hypertension in systemic lupus erythematosus","volume":"32","author":"Lian F","year":"2012","unstructured":"Lian F, Chen D, Wang Y, et al.. Clinical features and independent predictors of pulmonary arterial hypertension in systemic lupus erythematosus. Rheumatol Int. 2012, 32:1727-31. 10.1007\/s00296-011-1880-4","journal-title":"Rheumatol Int"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2004.02.029","article-title":"Cellular and molecular pathobiology of pulmonary arterial hypertension","volume":"43","author":"Humbert M","year":"2004","unstructured":"Humbert M, Morrell NW, Archer SL, et al.. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol. 2004, 43:13S-24S. 10.1016\/j.jacc.2004.02.029","journal-title":"J Am Coll Cardiol"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2004.02.033","article-title":"Pathologic assessment of vasculopathies in pulmonary hypertension","volume":"43","author":"Pietra GG","year":"2004","unstructured":"Pietra GG, Capron F, Stewart S, et al.. Pathologic assessment of vasculopathies in pulmonary hypertension. J Am Coll Cardiol. 2004, 43:25S-32S. 10.1016\/j.jacc.2004.02.033","journal-title":"J Am Coll Cardiol"},{"key":"ref22","doi-asserted-by":"publisher","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 S","year":"2015","unstructured":"Kommireddy S, Bhyravavajhala S, Kurimeti K, Chennareddy S, Kanchinadham S, Rajendra Vara Prasad I, Rajasekhar L. Pulmonary arterial hypertension in systemic lupus erythematosus may benefit by addition of immunosuppression to vasodilator therapy: an observational study. Rheumatology (Oxford). 2015, 54:1673-9. 10.1093\/rheumatology\/kev097","journal-title":"Rheumatology (Oxford)"},{"key":"ref23","doi-asserted-by":"publisher","DOI":"10.1080\/1744666X.2021.1825940","article-title":"Diagnostic and prognostic markers and treatment of connective tissue disease-associated pulmonary arterial hypertension: current recommendations and recent advances","volume":"16","author":"Kato M","year":"2020","unstructured":"Kato M, Sugimoto A, Atsumi T. Diagnostic and prognostic markers and treatment of connective tissue disease-associated pulmonary arterial hypertension: current recommendations and recent advances. Expert Rev Clin Immunol. 2020, 16:993-1004. 10.1080\/1744666X.2021.1825940","journal-title":"Expert Rev Clin Immunol"},{"key":"ref24","doi-asserted-by":"publisher","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 X","year":"2008","unstructured":"Jais X, Launay D, Yaici A, et al.. Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases. Arthritis Rheum. 2008, 58:521-31. 10.1002\/art.23303","journal-title":"Arthritis Rheum"},{"key":"ref25","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00003811","article-title":"Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus","volume":"20","author":"Johnson SR","year":"2011","unstructured":"Johnson SR, Granton JT. Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus. Eur Respir Rev. 2011, 20:277-86. 10.1183\/09059180.00003811","journal-title":"Eur Respir Rev"},{"key":"ref26","doi-asserted-by":"publisher","DOI":"10.1191\/0961203304lu509oa","article-title":"Therapy with intermittent pulse cyclophosphamide for pulmonary hypertension associated with systemic lupus erythematosus","volume":"13","author":"Gonzalez-Lopez L","year":"2004","unstructured":"Gonzalez-Lopez L, Cardona-Mu\u00f1oz EG, Celis A, et al.. Therapy with intermittent pulse cyclophosphamide for pulmonary hypertension associated with systemic lupus erythematosus. Lupus. 2004, 13:105-12. 10.1191\/0961203304lu509oa","journal-title":"Lupus"},{"key":"ref27","doi-asserted-by":"publisher","DOI":"10.1016\/0049-0172(84)90015-5","article-title":"Immunopathologic and clinical studies in pulmonary hypertension associated with SLE","volume":"13","author":"Quismorio FP Jr","year":"1984","unstructured":"Quismorio FP Jr, Sharma O, Koss M, Boylen T, Edmiston AW, Thornton PJ, Tatter D. Immunopathologic and clinical studies in pulmonary hypertension associated with SLE. Semin Arthritis Rheum. 1984, 13:349-59. 10.1016\/0049-0172(84)90015-5","journal-title":"Semin Arthritis Rheum"},{"key":"ref28","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200610-1559OC","article-title":"Role of endothelium-derived CC chemokine ligand 2 in idiopathic pulmonary arterial hypertension","volume":"176","author":"Sanchez O","year":"2007","unstructured":"Sanchez O, Marcos E, Perros F, et al.. Role of endothelium-derived CC chemokine ligand 2 in idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med. 2007, 176:1041-7. 10.1164\/rccm.200610-1559OC","journal-title":"Am J Respir Crit Care Med"},{"key":"ref29","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00104706","article-title":"Fractalkine-induced smooth muscle cell proliferation in pulmonary hypertension","volume":"29","author":"Perros F","year":"2007","unstructured":"Perros F, Dorfm\u00fcller P, Souza R, et al.. Fractalkine-induced smooth muscle cell proliferation in pulmonary hypertension. Eur Respir J. 2007, 29:937-43. 10.1183\/09031936.00104706","journal-title":"Eur Respir J"},{"key":"ref30","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2021.658341","article-title":"Exploration of the shared gene signatures and molecular mechanisms between systemic lupus erythematosus and pulmonary arterial hypertension: evidence from transcriptome data","volume":"12","author":"Yao M","year":"2021","unstructured":"Yao M, Zhang C, Gao C, et al.. Exploration of the shared gene signatures and molecular mechanisms between systemic lupus erythematosus and pulmonary arterial hypertension: evidence from transcriptome data. Front Immunol. 2021, 12:658341. 10.3389\/fimmu.2021.658341","journal-title":"Front Immunol"},{"key":"ref31","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201202-0335OC","article-title":"Immune and inflammatory cell involvement in the pathology of idiopathic pulmonary arterial hypertension","volume":"186","author":"Savai R","year":"2012","unstructured":"Savai R, Pullamsetti SS, Kolbe J, et al.. Immune and inflammatory cell involvement in the pathology of idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med. 2012, 186:897-908. 10.1164\/rccm.201202-0335OC","journal-title":"Am J Respir Crit Care Med"},{"key":"ref32","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCRESAHA.114.302221","article-title":"Evidence for the involvement of type I interferon in pulmonary arterial hypertension","volume":"114","author":"George PM","year":"2014","unstructured":"George PM, Oliver E, Dorfmuller P, et al.. Evidence for the involvement of type I interferon in pulmonary arterial hypertension. Circ Res. 2014, 114:677-88. 10.1161\/CIRCRESAHA.114.302221","journal-title":"Circ Res"},{"key":"ref33","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-017-3743-z","article-title":"Efficacy of immunosuppressive therapy on pulmonary arterial hypertension in systemic lupus erythematosus: a retrospective cohort study","volume":"36","author":"Ling YH","year":"2017","unstructured":"Ling YH, Fan YY, Yuan JJ, et al.. Efficacy of immunosuppressive therapy on pulmonary arterial hypertension in systemic lupus erythematosus: a retrospective cohort study. Clin Rheumatol. 2017, 36:2533-41. 10.1007\/s10067-017-3743-z","journal-title":"Clin Rheumatol"},{"key":"ref34","doi-asserted-by":"publisher","DOI":"10.12669\/pjms.35.1.405","article-title":"Frequency and predictors of pulmonary hypertension in patients with systemic lupus erythematosus","volume":"35","author":"Asif S","year":"2019","unstructured":"Asif S, Rasheed A, Mahmud TE, Asghar A. Frequency and predictors of pulmonary hypertension in patients with systemic lupus erythematosus. Pak J Med Sci. 2019, 35:86-9. 10.12669\/pjms.35.1.405","journal-title":"Pak J Med Sci"},{"key":"ref35","doi-asserted-by":"publisher","DOI":"10.1177\/0961203307087188","article-title":"An update in pulmonary hypertension in systemic lupus erythematosus - do we need to know about it?","volume":"17","author":"Pope J","year":"2008","unstructured":"Pope J. An update in pulmonary hypertension in systemic lupus erythematosus - do we need to know about it?. Lupus. 2008, 17:274-7. 10.1177\/0961203307087188","journal-title":"Lupus"},{"key":"ref36","doi-asserted-by":"publisher","DOI":"10.1016\/j.rcreu.2022.10.004","article-title":"Interstitial lung disease in systemic lupus erythematosus: state of the art search","volume":"31","author":"Santacruz JC","year":"2024","unstructured":"Santacruz JC, Mantilla MJ, Rodr\u00edguez-Salas G, Rueda I, Mar\u00eda Santos A, Londo\u00f1o J. Interstitial lung disease in systemic lupus erythematosus: state of the art search. Respir Case Rep. 2024, 31:234-42. 10.1016\/j.rcreu.2022.10.004","journal-title":"Respir Case Rep"},{"key":"ref37","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2014.10.012","article-title":"Risk of venous thromboembolism occurrence among adults with selected autoimmune diseases: a study among a U.S. cohort of commercial insurance enrollees","volume":"135","author":"Yusuf HR","year":"2015","unstructured":"Yusuf HR, Hooper WC, Grosse SD, Parker CS, Boulet SL, Ortel TL. Risk of venous thromboembolism occurrence among adults with selected autoimmune diseases: a study among a U.S. cohort of commercial insurance enrollees. Thromb Res. 2015, 135:50-7. 10.1016\/j.thromres.2014.10.012","journal-title":"Thromb Res"},{"key":"ref38","doi-asserted-by":"publisher","DOI":"10.1136\/rmdopen-2024-005330","article-title":"Prognostic insights from symptom clustering analysis in systemic lupus erythematosus","volume":"11","author":"Le Tallec E","year":"2025","unstructured":"Le Tallec E, Bourg C, Bouzill\u00e9 G, et al.. Prognostic insights from symptom clustering analysis in systemic lupus erythematosus. RMD Open. 2025, 11:005330. 10.1136\/rmdopen-2024-005330","journal-title":"RMD Open"},{"key":"ref39","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00879-2022","article-title":"2022 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"61","author":"Humbert M","year":"2023","unstructured":"Humbert M, Kovacs G, Hoeper MM, et al.. 2022 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2023, 61:2200879. 10.1183\/13993003.00879-2022","journal-title":"Eur Respir J"},{"key":"ref40","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00990-2016","article-title":"Exercise pulmonary haemodynamics predict outcome in patients with systemic sclerosis","volume":"48","author":"Stamm A","year":"2016","unstructured":"Stamm A, Saxer S, Lichtblau M, et al.. Exercise pulmonary haemodynamics predict outcome in patients with systemic sclerosis. Eur Respir J. 2016, 48:1658-67. 10.1183\/13993003.00990-2016","journal-title":"Eur Respir J"},{"key":"ref41","doi-asserted-by":"publisher","DOI":"10.1093\/ehjci\/jex244","article-title":"Standardization of adult transthoracic echocardiography reporting in agreement with recent chamber quantification, diastolic function, and heart valve disease recommendations: an expert consensus document of the European Association of Cardiovascular Imaging","volume":"18","author":"Galderisi M","year":"2017","unstructured":"Galderisi M, Cosyns B, Edvardsen T, et al.. Standardization of adult transthoracic echocardiography reporting in agreement with recent chamber quantification, diastolic function, and heart valve disease recommendations: an expert consensus document of the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging. 2017, 18:1301-10. 10.1093\/ehjci\/jex244","journal-title":"Eur Heart J Cardiovasc Imaging"},{"key":"ref42","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.104.488486","article-title":"Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension","volume":"111","author":"Sitbon O","year":"2005","unstructured":"Sitbon O, Humbert M, Ja\u00efs X, et al.. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005, 111:3105-11. 10.1161\/CIRCULATIONAHA.104.488486","journal-title":"Circulation"},{"key":"ref43","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehq170","article-title":"Long-term response to calcium-channel blockers in non-idiopathic pulmonary arterial hypertension","volume":"31","author":"Montani D","year":"2010","unstructured":"Montani D, Savale L, Natali D, et al.. Long-term response to calcium-channel blockers in non-idiopathic pulmonary arterial hypertension. Eur Heart J. 2010, 31:1898-907. 10.1093\/eurheartj\/ehq170","journal-title":"Eur Heart J"},{"key":"ref44","doi-asserted-by":"publisher","DOI":"10.1055\/s-0039-1685537","article-title":"Pulmonary complications of systemic lupus erythematosus","volume":"40","author":"Hannah JR","year":"2019","unstructured":"Hannah JR, D'Cruz DP. Pulmonary complications of systemic lupus erythematosus. Semin Respir Crit Care Med. 2019, 40:227-34. 10.1055\/s-0039-1685537","journal-title":"Semin Respir Crit Care Med"},{"key":"ref45","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2024.1374100","article-title":"Circulating extracellular vesicles as novel biomarkers for pulmonary arterial hypertension in patients with systemic lupus erythematosus","volume":"15","author":"Ding Z","year":"2024","unstructured":"Ding Z, Qi F, Liu L, et al.. Circulating extracellular vesicles as novel biomarkers for pulmonary arterial hypertension in patients with systemic lupus erythematosus. Front Immunol. 2024, 15:1374100. 10.3389\/fimmu.2024.1374100","journal-title":"Front Immunol"},{"key":"ref46","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-025-03169-x","article-title":"Deciphering the transcriptomic landscape of systemic lupus erythematosus-associated pulmonary arterial hypertension","volume":"26","author":"Li Y","year":"2025","unstructured":"Li Y, Qian J, Deng X, et al.. Deciphering the transcriptomic landscape of systemic lupus erythematosus-associated pulmonary arterial hypertension. Respir Res. 2025, 26:106. 10.1186\/s12931-025-03169-x","journal-title":"Respir Res"},{"key":"ref47","doi-asserted-by":"publisher","DOI":"10.1002\/art.39212","article-title":"Anti-endothelin receptor type A autoantibodies in systemic lupus erythematosus-associated pulmonary arterial hypertension","volume":"67","author":"Guo L","year":"2015","unstructured":"Guo L, Li M, Chen Y, et al.. Anti-endothelin receptor type A autoantibodies in systemic lupus erythematosus-associated pulmonary arterial hypertension. Arthritis Rheumatol. 2015, 67:2394-402. 10.1002\/art.39212","journal-title":"Arthritis Rheumatol"},{"key":"ref48","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2017.04.003","article-title":"Antiphospholipid antibodies can identify lupus patients at risk of pulmonary hypertension: a systematic review and meta-analysis","volume":"16","author":"Zuily S","year":"2017","unstructured":"Zuily S, Domingues V, Suty-Selton C, et al.. Antiphospholipid antibodies can identify lupus patients at risk of pulmonary hypertension: a systematic review and meta-analysis. Autoimmun Rev. 2017, 16:576-86. 10.1016\/j.autrev.2017.04.003","journal-title":"Autoimmun Rev"},{"key":"ref49","doi-asserted-by":"publisher","DOI":"10.1136\/lupus-2019-000356","article-title":"Autoantibody clustering of lupus-associated pulmonary hypertension","volume":"6","author":"Mizus M","year":"2019","unstructured":"Mizus M, Li J, Goldman D, Petri MA. Autoantibody clustering of lupus-associated pulmonary hypertension. Lupus Sci Med. 2019, 6:e000356. 10.1136\/lupus-2019-000356","journal-title":"Lupus Sci Med"},{"key":"ref50","doi-asserted-by":"publisher","DOI":"10.1111\/1756-185X.12262","article-title":"High levels of uric acid in systemic lupus erythematosus is associated with pulmonary hypertension","volume":"18","author":"Kim KJ","year":"2015","unstructured":"Kim KJ, Baek IW, Park YJ, Yoon CH, Kim WU, Cho CS. High levels of uric acid in systemic lupus erythematosus is associated with pulmonary hypertension. Int J Rheum Dis. 2015, 18:524-32. 10.1111\/1756-185X.12262","journal-title":"Int J Rheum Dis"},{"key":"ref51","doi-asserted-by":"publisher","DOI":"10.1177\/0961203315600539","article-title":"Levels of uric acid may predict the future development of pulmonary hypertension in systemic lupus erythematosus: a seven-year follow-up study","volume":"25","author":"Castillo-Mart\u00ednez D","year":"2016","unstructured":"Castillo-Mart\u00ednez D, Marroqu\u00edn-Fabi\u00e1n E, Lozada-Navarro AC, et al.. Levels of uric acid may predict the future development of pulmonary hypertension in systemic lupus erythematosus: a seven-year follow-up study. Lupus. 2016, 25:61-6. 10.1177\/0961203315600539","journal-title":"Lupus"},{"key":"ref52","doi-asserted-by":"publisher","DOI":"10.1177\/1759720X211040696","article-title":"Diagnosis and management of lung involvement in systemic lupus erythematosus and Sj\u00f6gren's syndrome: a literature review","volume":"13","author":"Depascale R","year":"2021","unstructured":"Depascale R, Del Frate G, Gasparotto M, et al.. Diagnosis and management of lung involvement in systemic lupus erythematosus and Sj\u00f6gren's syndrome: a literature review. Ther Adv Musculoskelet Dis. 2021, 13:1759720X211040696. 10.1177\/1759720X211040696","journal-title":"Ther Adv Musculoskelet Dis"},{"key":"ref53","doi-asserted-by":"publisher","DOI":"10.1111\/1756-185X.14706","article-title":"Immunosuppression for the treatment of pulmonary hypertension in patients with systemic lupus erythematosus: a\u00a0systematic review","volume":"26","author":"Bruera S","year":"2023","unstructured":"Bruera S, Sreedhar A, Agarwal SK, Muthukumar V, Geng Y, Lopez-Olivo MA. Immunosuppression for the treatment of pulmonary hypertension in patients with systemic lupus erythematosus: a\u00a0systematic review. Int J Rheum Dis. 2023, 26:1022-8. 10.1111\/1756-185X.14706","journal-title":"Int J Rheum Dis"},{"key":"ref54","article-title":"Clinical analysis of patients with systemic lupus erythematosus and concomitant pulmonary hypertension [Article in Chinese]","volume":"28","author":"Luo RQ","year":"2008","unstructured":"Luo RQ, Lei YX, Zhang X, Liang F. Clinical analysis of patients with systemic lupus erythematosus and concomitant pulmonary hypertension [Article in Chinese]. Nan Fang Yi Ke Da Xue Xue Bao. 2008, 28:1860-3.","journal-title":"Nan Fang Yi Ke Da Xue Xue Bao"},{"key":"ref55","doi-asserted-by":"publisher","DOI":"10.1177\/0961203307087610","article-title":"Rituximab treatment of pulmonary arterial hypertension associated with systemic lupus erythematosus: a case report","volume":"17","author":"Hennigan S","year":"2008","unstructured":"Hennigan S, Channick RN, Silverman GJ. Rituximab treatment of pulmonary arterial hypertension associated with systemic lupus erythematosus: a case report. Lupus. 2008, 17:754-6. 10.1177\/0961203307087610","journal-title":"Lupus"},{"key":"ref56","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2016-210236","article-title":"Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial","volume":"76","author":"Coghlan JG","year":"2017","unstructured":"Coghlan JG, Gali\u00e8 N, Barber\u00e0 JA, et al.. Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial. Ann Rheum Dis. 2017, 76:1219-27. 10.1136\/annrheumdis-2016-210236","journal-title":"Ann Rheum Dis"},{"key":"ref57","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1413687","article-title":"Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension","volume":"373","author":"Gali\u00e8 N","year":"2015","unstructured":"Gali\u00e8 N, Barber\u00e0 JA, Frost AE, et al.. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med. 2015, 373:834-44. 10.1056\/NEJMoa1413687","journal-title":"N Engl J Med"},{"key":"ref58","article-title":"Sildenafil for pulmonary arterial hypertension associated with connective tissue disease","volume":"34","author":"Badesch DB","year":"2007","unstructured":"Badesch DB, Hill NS, Burgess G, Rubin LJ, Barst RJ, Gali\u00e8 N, Simonneau G. Sildenafil for pulmonary arterial hypertension associated with connective tissue disease. J Rheumatol. 2007, 34:2417-22.","journal-title":"J Rheumatol"},{"key":"ref59","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01325-2024","article-title":"Treatment algorithm for pulmonary arterial hypertension","volume":"64","author":"Chin KM","year":"2024","unstructured":"Chin KM, Gaine SP, Gerges C, et al.. Treatment algorithm for pulmonary arterial hypertension. Eur Respir J. 2024, 64:2400187. 10.1183\/13993003.01325-2024","journal-title":"Eur Respir J"},{"key":"ref60","doi-asserted-by":"publisher","DOI":"10.1378\/chest.117.1.14","article-title":"Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus","volume":"117","author":"Robbins IM","year":"2000","unstructured":"Robbins IM, Gaine SP, Schilz R, Tapson VF, Rubin LJ, Loyd JE. Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus. Chest. 2000, 117:14-18. 10.1378\/chest.117.1.14","journal-title":"Chest"},{"key":"ref61","doi-asserted-by":"publisher","DOI":"10.1378\/chest.126.2.420","article-title":"Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease","volume":"126","author":"Oudiz RJ","year":"2004","unstructured":"Oudiz RJ, Schilz RJ, Barst RJ, Gali\u00e9 N, Rich S, Rubin LJ, Simonneau G. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest. 2004, 126:420-7. 10.1378\/chest.126.2.420","journal-title":"Chest"},{"key":"ref62","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2213558","article-title":"Phase 3 trial of sotatercept for treatment of pulmonary arterial hypertension","volume":"388","author":"Hoeper MM","year":"2023","unstructured":"Hoeper MM, Badesch DB, Ghofrani HA, et al.. Phase 3 trial of sotatercept for treatment of pulmonary arterial hypertension. N Engl J Med. 2023, 388:1478-90. 10.1056\/NEJMoa2213558","journal-title":"N Engl J Med"},{"key":"ref63","doi-asserted-by":"publisher","DOI":"10.3390\/jcm14155177","article-title":"Sotatercept for connective tissue disease-associated pulmonary arterial hypertension with concomitant interstitial lung disease: efficacy and safety insights","volume":"14","author":"Dagher C","year":"2025","unstructured":"Dagher C, Akiki M, Swanson K, Carollo B, Fiscus G, Farber HW, Parikh R. Sotatercept for connective tissue disease-associated pulmonary arterial hypertension with concomitant interstitial lung disease: efficacy and safety insights. J Clin Med. 2025, 14:5177. 10.3390\/jcm14155177","journal-title":"J Clin Med"},{"key":"ref64","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2017.08.014","article-title":"Pulmonary arterial hypertension associated with systemic lupus erythematosus: results from the French Pulmonary Hypertension Registry","volume":"153","author":"Hachulla E","year":"2018","unstructured":"Hachulla E, Jais X, Cinquetti G, et al.. Pulmonary arterial hypertension associated with systemic lupus erythematosus: results from the French Pulmonary Hypertension Registry. Chest. 2018, 153:143-51. 10.1016\/j.chest.2017.08.014","journal-title":"Chest"},{"key":"ref65","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2015.11.012","article-title":"Survival and prognostic factors of systemic lupus erythematosus-associated pulmonary arterial hypertension: a PRISMA-compliant systematic review and meta-analysis","volume":"15","author":"Qian J","year":"2016","unstructured":"Qian J, Wang Y, Huang C, et al.. Survival and prognostic factors of systemic lupus erythematosus-associated pulmonary arterial hypertension: a PRISMA-compliant systematic review and meta-analysis. Autoimmun Rev. 2016, 15:250-7. 10.1016\/j.autrev.2015.11.012","journal-title":"Autoimmun Rev"},{"key":"ref66","doi-asserted-by":"publisher","DOI":"10.3904\/kjim.2015.30.2.232","article-title":"Pulmonary hypertension in systemic lupus erythematosus: an independent predictor of patient survival","volume":"30","author":"Min HK","year":"2015","unstructured":"Min HK, Lee JH, Jung SM, et al.. Pulmonary hypertension in systemic lupus erythematosus: an independent predictor of patient survival. Korean J Intern Med. 2015, 30:232-41. 10.3904\/kjim.2015.30.2.232","journal-title":"Korean J Intern Med"}],"container-title":["Cureus"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cureus.com\/articles\/430787-pulmonary-hypertension-in-systemic-lupus-erythematosus-current-evidence-and-future-directions","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,26]],"date-time":"2025-11-26T15:55:32Z","timestamp":1764172532000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cureus.com\/articles\/430787-pulmonary-hypertension-in-systemic-lupus-erythematosus-current-evidence-and-future-directions"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,26]]},"references-count":66,"URL":"https:\/\/doi.org\/10.7759\/cureus.97863","relation":{},"ISSN":["2168-8184"],"issn-type":[{"value":"2168-8184","type":"print"}],"subject":[],"published":{"date-parts":[[2025,11,26]]}}}