{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T05:56:13Z","timestamp":1790834173713,"version":"4.1.0"},"reference-count":109,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,1,10]],"date-time":"2025-01-10T00:00:00Z","timestamp":1736467200000},"content-version":"am","delay-in-days":375,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100004330","name":"GlaxoSmithKline","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004330","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100014255","name":"Fonds de dotation CSL Behring pour la recherche","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100014255","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.fr","em-consulte.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["La Revue de M\u00e9decine Interne"],"published-print":{"date-parts":[[2024,1]]},"DOI":"10.1016\/j.revmed.2023.10.450","type":"journal-article","created":{"date-parts":[[2023,11,2]],"date-time":"2023-11-02T18:15:46Z","timestamp":1698948946000},"page":"26-40","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":3,"title":["Pulmonary hypertension in connective tissue diseases: What every CTD specialist should know\u00a0\u2013\u00a0but is afraid to ask!"],"prefix":"10.1016","volume":"45","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0280-411X","authenticated-orcid":false,"given":"S.","family":"Sanges","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V.","family":"Sobanski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"Lamblin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Hachulla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Savale","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9358-6922","authenticated-orcid":false,"given":"D.","family":"Montani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Launay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.revmed.2023.10.450_bib0550","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1111\/j.1398-9995.2005.00761.x","article-title":"The respiratory system in connective tissue disorders","volume":"60","author":"Crestani","year":"2005","journal-title":"Allergy"},{"key":"10.1016\/j.revmed.2023.10.450_bib0555","series-title":"Primary pulmonary hypertension: report on a WHO meeting","author":"Hatano","year":"1975"},{"key":"10.1016\/j.revmed.2023.10.450_bib0560","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1183\/09031936.00008611","article-title":"Pulmonary vascular resistances during exercise in normal subjects: a systematic review","volume":"39","author":"Kovacs","year":"2012","journal-title":"Eur Respir J"},{"key":"10.1016\/j.revmed.2023.10.450_bib0565","doi-asserted-by":"crossref","first-page":"2200879","DOI":"10.1183\/13993003.00879-2022","article-title":"2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"61","author":"Humbert","year":"2023","journal-title":"Eur Respir J"},{"key":"10.1016\/j.revmed.2023.10.450_bib0570","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1183\/16000617.0062-2015","article-title":"Right heart catheterisation: best practice and pitfalls in pulmonary hypertension","volume":"24","author":"Rosenkranz","year":"2015","journal-title":"Eur Respir Rev"},{"key":"10.1016\/j.revmed.2023.10.450_bib0575","doi-asserted-by":"crossref","first-page":"1801897","DOI":"10.1183\/13993003.01897-2018","article-title":"Pulmonary hypertension due to left heart disease","volume":"53","author":"Vachi\u00e9ry","year":"2019","journal-title":"Eur Respir J"},{"key":"10.1016\/j.revmed.2023.10.450_bib0580","doi-asserted-by":"crossref","first-page":"1898","DOI":"10.1093\/eurheartj\/ehq170","article-title":"Long-term response to calcium-channel blockers in non-idiopathic pulmonary arterial hypertension","volume":"31","author":"Montani","year":"2010","journal-title":"Eur Heart J"},{"key":"10.1016\/j.revmed.2023.10.450_bib0585","doi-asserted-by":"crossref","first-page":"170056","DOI":"10.1183\/16000617.0056-2017","article-title":"Pulmonary hypertension in systemic sclerosis: different phenotypes","volume":"26","author":"Launay","year":"2017","journal-title":"Eur Respir Rev"},{"key":"10.1016\/j.revmed.2023.10.450_bib0590","doi-asserted-by":"crossref","first-page":"2101483","DOI":"10.1183\/13993003.01483-2021","article-title":"Pulmonary vascular resistance predicts mortality in patients with pulmonary hypertension associated with interstitial lung disease: results from the COMPERA registry","volume":"58","author":"Olsson","year":"2021","journal-title":"Eur Respir J"},{"key":"10.1016\/j.revmed.2023.10.450_bib0595","doi-asserted-by":"crossref","first-page":"2995","DOI":"10.1002\/art.34501","article-title":"Computed tomography findings of pulmonary venoocclusive disease in scleroderma patients presenting with precapillary pulmonary hypertension","volume":"64","author":"G\u00fcnther","year":"2012","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.revmed.2023.10.450_bib0600","doi-asserted-by":"crossref","first-page":"1518","DOI":"10.1183\/13993003.00026-2016","article-title":"Pulmonary veno-occlusive disease","volume":"47","author":"Montani","year":"2016","journal-title":"Eur Respir J"},{"key":"10.1016\/j.revmed.2023.10.450_bib0605","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1007\/s12016-010-8198-y","article-title":"Diagnosis and classification of systemic sclerosis","volume":"40","author":"Hachulla","year":"2011","journal-title":"Clin Rev Allergy Immunol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0610","doi-asserted-by":"crossref","first-page":"940","DOI":"10.1136\/ard.2006.066068","article-title":"Changes in causes of death in systemic sclerosis, 1972\u20132002","volume":"66","author":"Steen","year":"2007","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.revmed.2023.10.450_bib0615","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.1164\/rccm.200510-1668OC","article-title":"Pulmonary arterial hypertension in France: results from a national registry","volume":"173","author":"Humbert","year":"2006","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.revmed.2023.10.450_bib0620","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1164\/rccm.200806-953OC","article-title":"Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era","volume":"179","author":"Condliffe","year":"2009","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.revmed.2023.10.450_bib0625","doi-asserted-by":"crossref","first-page":"1383","DOI":"10.1378\/chest.10-0260","article-title":"Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL","volume":"138","author":"Chung","year":"2010","journal-title":"Chest"},{"key":"10.1016\/j.revmed.2023.10.450_bib0630","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1183\/09031936.00106008","article-title":"Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: a distinctive vasculopathy","volume":"34","author":"Overbeek","year":"2009","journal-title":"Eur Respir J"},{"key":"10.1016\/j.revmed.2023.10.450_bib0635","doi-asserted-by":"crossref","first-page":"1801887","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":"10.1016\/j.revmed.2023.10.450_bib0640","doi-asserted-by":"crossref","first-page":"1285","DOI":"10.1164\/rccm.200909-1331PP","article-title":"Systemic sclerosis-associated pulmonary arterial hypertension","volume":"181","author":"Le Pavec","year":"2010","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.revmed.2023.10.450_bib0645","doi-asserted-by":"crossref","first-page":"434","DOI":"10.1016\/S0046-8177(97)90032-0","article-title":"Pathogenesis and evolution of plexiform lesions in pulmonary hypertension associated with scleroderma and human immunodeficiency virus infection","volume":"28","author":"Cool","year":"1997","journal-title":"Hum Pathol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0650","doi-asserted-by":"crossref","first-page":"893","DOI":"10.1016\/j.humpath.2006.11.022","article-title":"Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases","volume":"38","author":"Dorfm\u00fcller","year":"2007","journal-title":"Hum Pathol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0655","doi-asserted-by":"crossref","first-page":"954007","DOI":"10.3389\/fimmu.2022.954007","article-title":"Soluble markers of B cell activation suggest a role of B cells in the pathogenesis of systemic sclerosis-associated pulmonary arterial hypertension","volume":"13","author":"Sanges","year":"2022","journal-title":"Front Immunol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0660","doi-asserted-by":"crossref","first-page":"930970","DOI":"10.3389\/fimmu.2022.930970","article-title":"Can antinuclear antibodies have a pathogenic role in systemic sclerosis?","volume":"13","author":"Chepy","year":"2022","journal-title":"Front Immunol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0665","doi-asserted-by":"crossref","first-page":"1016","DOI":"10.1378\/chest.10-2473","article-title":"Clinical characteristics and survival in systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease","volume":"140","author":"Launay","year":"2011","journal-title":"Chest"},{"key":"10.1016\/j.revmed.2023.10.450_bib0670","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1016\/j.semarthrit.2018.10.011","article-title":"Combined pulmonary fibrosis and emphysema in systemic sclerosis: a syndrome associated with heavy morbidity and mortality","volume":"49","author":"Champtiaux","year":"2019","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.revmed.2023.10.450_bib0675","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1002\/acr.22673","article-title":"The risk of pulmonary embolism and deep venous thrombosis in systemic sclerosis: a general population-based study","volume":"68","author":"Schoenfeld","year":"2016","journal-title":"Arthritis Care Res"},{"key":"10.1016\/j.revmed.2023.10.450_bib0680","doi-asserted-by":"crossref","first-page":"188","DOI":"10.28982\/josam.841679","article-title":"Experience of chronic thromboembolic pulmonary hypertension (CTEPH) in two cases with scleroderma and immunopathogenesis overview: case report","volume":"5","author":"Okyar","year":"2021","journal-title":"J Surg Med"},{"key":"10.1016\/j.revmed.2023.10.450_bib0685","doi-asserted-by":"crossref","first-page":"ytac080","DOI":"10.1093\/ehjcr\/ytac080","article-title":"Systemic sclerosis complicated by chronic thromboembolic pulmonary hypertension treated with balloon pulmonary angioplasty: a case report","volume":"6","author":"Matsui","year":"2022","journal-title":"Eur Heart J Case Rep"},{"key":"10.1016\/j.revmed.2023.10.450_bib0690","doi-asserted-by":"crossref","first-page":"e0197112","DOI":"10.1371\/journal.pone.0197112","article-title":"Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis","volume":"13","author":"Launay","year":"2018","journal-title":"PLoS One"},{"key":"10.1016\/j.revmed.2023.10.450_bib0695","article-title":"Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study","author":"Coghlan","year":"2014731340","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.revmed.2023.10.450_bib0700","doi-asserted-by":"crossref","first-page":"2290","DOI":"10.3899\/jrheum.100245","article-title":"Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and meta-analysis of 5 studies","volume":"37","author":"Avouac","year":"2010","journal-title":"J Rheumatol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0705","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1136\/annrheumdis-2019-216476","article-title":"Haemodynamic phenotypes and survival in patients with systemic sclerosis: the impact of the new definition of pulmonary arterial hypertension","volume":"79","author":"Xanthouli","year":"2020","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.revmed.2023.10.450_bib0710","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1186\/s12931-022-02205-4","article-title":"Mild pulmonary hemodynamic alterations in patients with systemic sclerosis: relevance of the new 2022 ESC\/ERS definition of pulmonary hypertension and impact on mortality","volume":"23","author":"Puigrenier","year":"2022","journal-title":"Respir Res"},{"key":"10.1016\/j.revmed.2023.10.450_bib0715","doi-asserted-by":"crossref","first-page":"1831","DOI":"10.1002\/art.24525","article-title":"The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France","volume":"60","author":"Hachulla","year":"2009","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.revmed.2023.10.450_bib0720","doi-asserted-by":"crossref","first-page":"102602","DOI":"10.1016\/j.autrev.2020.102602","article-title":"Factors associated with pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc)","volume":"19","author":"Jiang","year":"2020","journal-title":"Autoimmun Rev"},{"key":"10.1016\/j.revmed.2023.10.450_bib0725","doi-asserted-by":"crossref","first-page":"1211","DOI":"10.1378\/chest.08-3042","article-title":"Is pulmonary arterial hypertension really a late complication of systemic sclerosis?","volume":"136","author":"Hachulla","year":"2009","journal-title":"Chest"},{"key":"10.1016\/j.revmed.2023.10.450_bib0730","doi-asserted-by":"crossref","first-page":"2412","DOI":"10.1002\/art.38029","article-title":"Survival and prognostic factors in systemic sclerosis-associated pulmonary hypertension: a systematic review and meta-analysis: survival and prognosis in SSc-associated pulmonary hypertension","volume":"65","author":"Lef\u00e8vre","year":"2013","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.revmed.2023.10.450_bib0735","article-title":"Survival improved in patients aged \u2264 70 years with systemic sclerosis-associated pulmonary arterial hypertension during the period 2006 to 2017 in France","author":"Hachulla","year":"2020157945","journal-title":"Chest"},{"key":"10.1016\/j.revmed.2023.10.450_bib0740","doi-asserted-by":"crossref","first-page":"1940","DOI":"10.1136\/annrheumdis-2012-202489","article-title":"Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era","volume":"72","author":"Launay","year":"2013","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.revmed.2023.10.450_bib0745","doi-asserted-by":"crossref","first-page":"963","DOI":"10.1183\/09031936.00182813","article-title":"Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients","volume":"44","author":"Hao","year":"2014","journal-title":"Eur Respir J"},{"key":"10.1016\/j.revmed.2023.10.450_bib0750","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1016\/j.revmed.2011.01.002","article-title":"[Pulmonary arterial hypertension in systemic lupus erythematosus]","volume":"32","author":"Arnaud","year":"2011","journal-title":"Rev Med Interne"},{"key":"10.1016\/j.revmed.2023.10.450_bib0755","doi-asserted-by":"crossref","first-page":"143","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","year":"2018","journal-title":"Chest"},{"key":"10.1016\/j.revmed.2023.10.450_bib0760","doi-asserted-by":"crossref","first-page":"1403","DOI":"10.1055\/s-0039-1692428","article-title":"Antiphospholipid syndrome in chronic thromboembolic pulmonary hypertension: a well-defined subgroup of patients","volume":"119","author":"Jiang","year":"2019","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.revmed.2023.10.450_bib0765","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1016\/j.berh.2013.07.011","article-title":"Pulmonary hypertension in systemic lupus erythematosus","volume":"27","author":"Schreiber","year":"2013","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0770","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1016\/j.autrev.2012.07.010","article-title":"Pulmonary hypertension in systemic lupus erythematosus: prevalence, predictors and diagnostic strategy","volume":"12","author":"Ruiz-Irastorza","year":"2013","journal-title":"Autoimmun Rev"},{"key":"10.1016\/j.revmed.2023.10.450_bib0775","doi-asserted-by":"crossref","first-page":"250","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","year":"2016","journal-title":"Autoimmun Rev"},{"key":"10.1016\/j.revmed.2023.10.450_bib0780","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":"10.1016\/j.revmed.2023.10.450_bib0785","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1002\/art.23303","article-title":"Immunosuppressive therapy in lupus- and mixed connective tissue disease\u2013associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases","volume":"58","author":"Jais","year":"2008","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.revmed.2023.10.450_bib0790","doi-asserted-by":"crossref","first-page":"1322","DOI":"10.1002\/ehf2.12507","article-title":"Efficacy of immunosuppressants with bridge vasodilator therapy in severe lupus erythematosus-associated pulmonary arterial hypertension","volume":"6","author":"Sanges","year":"2019","journal-title":"ESC Heart Fail"},{"key":"10.1016\/j.revmed.2023.10.450_bib0795","doi-asserted-by":"crossref","first-page":"1208","DOI":"10.1093\/rheumatology\/kes430","article-title":"Prevalence of pulmonary hypertension in an unselected, mixed connective tissue disease cohort: results of a nationwide, Norwegian cross-sectional multicentre study and review of current literature","volume":"52","author":"Gunnarsson","year":"2013","journal-title":"Rheumatology"},{"key":"10.1016\/j.revmed.2023.10.450_bib0800","doi-asserted-by":"crossref","first-page":"3261","DOI":"10.1093\/rheumatology\/kead055","article-title":"In-depth characterization of pulmonary arterial hypertension in mixed connective tissue disease: a French national multicentre study","volume":"62","author":"Chaigne","year":"2023","journal-title":"Rheumatology"},{"key":"10.1016\/j.revmed.2023.10.450_bib0805","doi-asserted-by":"crossref","first-page":"1134","DOI":"10.3899\/jrheum.121272","article-title":"Clinical course, prognosis, and causes of death in mixed connective tissue disease","volume":"40","author":"Hajas","year":"2013","journal-title":"J Rheumatol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0810","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1002\/art.39432","article-title":"Characteristics and survival of anti-U1 RNP antibody-positive patients with connective tissue disease-associated pulmonary arterial hypertension","volume":"68","author":"Sobanski","year":"2016","journal-title":"Arthritis Rheumatol Hoboken NJ"},{"key":"10.1016\/j.revmed.2023.10.450_bib0815","doi-asserted-by":"crossref","first-page":"5115","DOI":"10.3390\/jcm10215115","article-title":"Cardiovascular events, sleep apnoea, and pulmonary hypertension in primary Sj\u00f6gren's syndrome: data from the French Health Insurance Database","volume":"10","author":"Goulabchand","year":"2021","journal-title":"J Clin Med"},{"key":"10.1016\/j.revmed.2023.10.450_bib0820","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1097\/MD.0b013e3181579781","article-title":"Pulmonary arterial hypertension: a rare complication of primary Sj\u00f6gren syndrome: report of 9 new cases and review of the literature","volume":"86","author":"Launay","year":"2007","journal-title":"Medicine (Baltimore)"},{"key":"10.1016\/j.revmed.2023.10.450_bib0825","doi-asserted-by":"crossref","first-page":"193","DOI":"10.3109\/14397595.2015.1059989","article-title":"Combined disease with pulmonary arterial hypertension and pulmonary venous hypertension revealed after treatment of heart failure with preserved ejection fraction in a case with primary Sj\u00f6gren syndrome","volume":"28","author":"Yamasaki","year":"2018","journal-title":"Mod Rheumatol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0830","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1186\/s12890-023-02322-w","article-title":"Pulmonary veno-occlusive disease in Sjogren's syndrome: a case report","volume":"23","author":"Zeng","year":"2023","journal-title":"BMC Pulm Med"},{"key":"10.1016\/j.revmed.2023.10.450_bib0835","doi-asserted-by":"crossref","first-page":"1902157","DOI":"10.1183\/13993003.02157-2019","article-title":"Pulmonary arterial hypertension associated with primary Sj\u00f6gren's syndrome: a multicentre cohort study from China","volume":"56","author":"Wang","year":"2020","journal-title":"Eur Respir J"},{"key":"10.1016\/j.revmed.2023.10.450_bib0840","doi-asserted-by":"crossref","first-page":"1068","DOI":"10.1111\/1756-185X.13290","article-title":"Characteristics and risk factors of pulmonary arterial hypertension in patients with primary Sj\u00f6gren's syndrome","volume":"21","author":"Yan","year":"2018","journal-title":"Int J Rheum Dis"},{"key":"10.1016\/j.revmed.2023.10.450_bib0845","doi-asserted-by":"crossref","first-page":"e4911","DOI":"10.1097\/MD.0000000000004911","article-title":"Pulmonary arterial hypertension in idiopathic inflammatory myopathies: data from the French pulmonary hypertension registry and review of the literature","volume":"95","author":"Sanges","year":"2016","journal-title":"Medicine (Baltimore)"},{"key":"10.1016\/j.revmed.2023.10.450_bib0850","doi-asserted-by":"crossref","first-page":"e000331","DOI":"10.1136\/rmdopen-2016-000331","article-title":"Pulmonary arterial hypertension, a novelty in idiopathic inflammatory myopathies: insights and first experiences with vasoactive therapy","volume":"3","author":"Bhansing","year":"2017","journal-title":"RMD Open"},{"key":"10.1016\/j.revmed.2023.10.450_bib0855","doi-asserted-by":"crossref","first-page":"1381","DOI":"10.1007\/s10067-010-1465-8","article-title":"Clinical characteristics and outcomes of patients with idiopathic inflammatory myopathies from Jordan 1996\u20132009","volume":"29","author":"Mustafa","year":"2010","journal-title":"Clin Rheumatol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0860","doi-asserted-by":"crossref","first-page":"2105","DOI":"10.1007\/s10067-015-3095-7","article-title":"Pulmonary hypertension in polymyositis","volume":"34","author":"Wang","year":"2015","journal-title":"Clin Rheumatol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0865","doi-asserted-by":"crossref","first-page":"1271","DOI":"10.1183\/09031936.00156312","article-title":"Pulmonary hypertension in antisynthetase syndrome: prevalence, aetiology and survival","volume":"42","author":"Hervier","year":"2013","journal-title":"Eur Respir J"},{"key":"10.1016\/j.revmed.2023.10.450_bib0870","first-page":"244","article-title":"Association between rheumatoid arthritis and pulmonary hypertension: data from the French Pulmonary Hypertension Registry","volume":"95","author":"Montani","year":"2018","journal-title":"Respir Int Rev Thorac Dis"},{"key":"10.1016\/j.revmed.2023.10.450_bib0875","doi-asserted-by":"crossref","first-page":"1306","DOI":"10.1513\/AnnalsATS.202008-913OC","article-title":"Association between leflunomide and pulmonary hypertension","volume":"18","author":"Lacoste-Palasset","year":"2021","journal-title":"Ann Am Thorac Soc"},{"key":"10.1016\/j.revmed.2023.10.450_bib0880","doi-asserted-by":"crossref","first-page":"G11","DOI":"10.1530\/ERP-17-0071","article-title":"Echocardiographic assessment of pulmonary hypertension: a guideline protocol from the British Society of Echocardiography","volume":"5","author":"Augustine","year":"2018","journal-title":"Echo Res Pract"},{"key":"10.1016\/j.revmed.2023.10.450_bib0885","doi-asserted-by":"crossref","first-page":"103290","DOI":"10.1016\/j.autrev.2023.103290","article-title":"The role of TAPSE\/sPAP ratio in predicting pulmonary hypertension and mortality in the systemic sclerosis EUSTAR cohort","volume":"22","author":"Colalillo","year":"2023","journal-title":"Autoimmun Rev"},{"key":"10.1016\/j.revmed.2023.10.450_bib0890","doi-asserted-by":"crossref","first-page":"1028","DOI":"10.1016\/S0735-1097(02)02964-9","article-title":"Pulmonary function in primary pulmonary hypertension","volume":"41","author":"Sun","year":"2003","journal-title":"J Am Coll Cardiol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0895","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1002\/art.42349","article-title":"Dynamic prediction of pulmonary hypertension in systemic sclerosis using landmark analysis","volume":"75","author":"Nihtyanova","year":"2023","journal-title":"Arthritis Rheumatol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0900","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1097\/MD.0b013e31818193bb","article-title":"Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology","volume":"87","author":"Montani","year":"2008","journal-title":"Medicine (Baltimore)"},{"key":"10.1016\/j.revmed.2023.10.450_bib0905","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/j.healun.2009.07.005","article-title":"Carbon monoxide diffusing capacity and mortality in pulmonary arterial hypertension","volume":"29","author":"Chandra","year":"2010","journal-title":"J Heart Lung Transplant"},{"key":"10.1016\/j.revmed.2023.10.450_bib0910","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1007\/s11739-016-1573-9","article-title":"Utility of FVC\/DLCO ratio to stratify the risk of mortality in unselected subjects with pulmonary hypertension","volume":"12","author":"Donato","year":"2017","journal-title":"Intern Emerg Med"},{"key":"10.1016\/j.revmed.2023.10.450_bib0915","doi-asserted-by":"crossref","first-page":"R143","DOI":"10.1186\/ar3876","article-title":"N-terminal pro-brain natriuretic peptide in a novel screening algorithm for pulmonary arterial hypertension in systemic sclerosis: a case-control study","volume":"14","author":"Thakkar","year":"2012","journal-title":"Arthritis Res Ther"},{"key":"10.1016\/j.revmed.2023.10.450_bib0920","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1164\/ajrccm.166.1.at1102","article-title":"ATS statement: guidelines for the six-minute walk test","volume":"166","year":"2002","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.revmed.2023.10.450_bib0925","doi-asserted-by":"crossref","first-page":"1457","DOI":"10.1136\/annrheumdis-2015-207336","article-title":"A prospective study of the 6 min walk test as a surrogate marker for haemodynamics in two independent cohorts of treatment-na\u00efve systemic sclerosis-associated pulmonary arterial hypertension","volume":"75","author":"Sanges","year":"2016","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.revmed.2023.10.450_bib0930","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1186\/s13075-017-1489-4","article-title":"Factors associated with the 6-minute walk distance in patients with systemic sclerosis","volume":"19","author":"Sanges","year":"2017","journal-title":"Arthritis Res Ther"},{"key":"10.1016\/j.revmed.2023.10.450_bib0935","doi-asserted-by":"crossref","first-page":"H2150","DOI":"10.1152\/ajpheart.01312.2007","article-title":"Improved ECG detection of presence and severity of right ventricular pressure load validated with cardiac magnetic resonance imaging","volume":"294","author":"Henkens","year":"2008","journal-title":"Am J Physiol Heart Circ Physiol"},{"key":"10.1016\/j.revmed.2023.10.450_bib0940","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1148\/radiol.2020203108","article-title":"Imaging of pulmonary hypertension in adults: a position paper from the Fleischner Society","volume":"298","author":"Remy-Jardin","year":"2021","journal-title":"Radiology"},{"key":"10.1016\/j.revmed.2023.10.450_bib0945","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1186\/s13023-021-01844-y","article-title":"French recommendations for the management of systemic sclerosis","volume":"16","author":"Hachulla","year":"2021","journal-title":"Orphanet J Rare Dis"},{"key":"10.1016\/j.revmed.2023.10.450_bib0950","doi-asserted-by":"crossref","first-page":"3194","DOI":"10.1002\/art.38172","article-title":"Recommendations for screening and detection of connective tissue disease-associated pulmonary arterial hypertension","volume":"65","author":"Khanna","year":"2013","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.revmed.2023.10.450_bib0955","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1016\/j.revmed.2017.05.003","article-title":"[Pulmonary hypertension screening in systemic sclerosis: the best strategy remains to be defined]","volume":"38","author":"Launay","year":"2017","journal-title":"Rev Med Interne"},{"key":"10.1016\/j.revmed.2023.10.450_bib0960","doi-asserted-by":"crossref","first-page":"2102419.","DOI":"10.1183\/13993003.02419-2021","article-title":"External validation of a refined four-stratum risk assessment score from the French pulmonary hypertension registry","volume":"59","author":"Boucly","year":"2022","journal-title":"Eur Respir J"},{"key":"10.1016\/j.revmed.2023.10.450_bib0965","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":"10.1016\/j.revmed.2023.10.450_bib0970","doi-asserted-by":"crossref","first-page":"2403","DOI":"10.1161\/CIRCULATIONAHA.115.018435","article-title":"Effect of warfarin treatment on survival of patients with pulmonary arterial hypertension (PAH) in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL)","volume":"132","author":"Preston","year":"2015","journal-title":"Circulation"},{"key":"10.1016\/j.revmed.2023.10.450_bib0975","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1161\/CIRCULATIONAHA.113.004526","article-title":"Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA)","volume":"129","author":"Olsson","year":"2014","journal-title":"Circulation"},{"key":"10.1016\/j.revmed.2023.10.450_bib0980","doi-asserted-by":"crossref","first-page":"1336","DOI":"10.1136\/ard.2005.048967","article-title":"Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions","volume":"65","author":"Denton","year":"2006","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.revmed.2023.10.450_bib0985","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1093\/rheumatology\/kep398","article-title":"Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil","volume":"49","author":"Launay","year":"2010","journal-title":"Rheumatol Oxf Engl"},{"key":"10.1016\/j.revmed.2023.10.450_bib0990","doi-asserted-by":"crossref","first-page":"488B","DOI":"10.1378\/chest.132.4_MeetingAbstracts.488b","article-title":"Ambrisentan therapy for pulmonary arterial hypertension: a comparison by PAH etiology","volume":"132","author":"Badesch","year":"2007","journal-title":"Chest"},{"key":"10.1016\/j.revmed.2023.10.450_bib0995","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1111\/j.1755-5922.2011.00279.x","article-title":"ARIES-3: ambrisentan therapy in a diverse population of patients with pulmonary hypertension","volume":"30","author":"Badesch","year":"2012","journal-title":"Cardiovasc Ther"},{"key":"10.1016\/j.revmed.2023.10.450_bib1000","first-page":"2417","article-title":"Sildenafil for pulmonary arterial hypertension associated with connective tissue disease","volume":"34","author":"Badesch","year":"2007","journal-title":"J Rheumatol"},{"key":"10.1016\/j.revmed.2023.10.450_bib1005","doi-asserted-by":"crossref","first-page":"2894","DOI":"10.1161\/CIRCULATIONAHA.108.839274","article-title":"Tadalafil therapy for pulmonary arterial hypertension","volume":"119","author":"Gali\u00e8","year":"2009","journal-title":"Circulation"},{"key":"10.1016\/j.revmed.2023.10.450_bib1010","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1136\/annrheumdis-2015-209087","article-title":"Riociguat for the treatment of pulmonary arterial hypertension associated with connective tissue disease: results from PATENT-1 and PATENT-2","volume":"76","author":"Humbert","year":"2017","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.revmed.2023.10.450_bib1015","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1164\/rccm.201507-1398OC","article-title":"Ambrisentan and tadalafil up-front combination therapy in scleroderma-associated pulmonary arterial hypertension","volume":"192","author":"Hassoun","year":"2015","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.revmed.2023.10.450_bib1020","doi-asserted-by":"crossref","first-page":"1219","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","year":"2017","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.revmed.2023.10.450_bib1025","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":"10.1016\/j.revmed.2023.10.450_bib1030","doi-asserted-by":"crossref","first-page":"420","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","year":"2004","journal-title":"Chest"},{"key":"10.1016\/j.revmed.2023.10.450_bib1035","doi-asserted-by":"crossref","first-page":"1602493","DOI":"10.1183\/13993003.02493-2016","article-title":"Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension","volume":"50","author":"Gaine","year":"2017","journal-title":"Eur Respir J"},{"key":"10.1016\/j.revmed.2023.10.450_bib1040","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1111\/1756-185X.14368","article-title":"Immunosuppressive therapy in patients with connective tissue disease-associated pulmonary arterial hypertension: a systematic review","volume":"25","author":"Ding","year":"2022","journal-title":"Int J Rheum Dis"},{"key":"10.1016\/j.revmed.2023.10.450_bib1045","doi-asserted-by":"crossref","first-page":"1602449","DOI":"10.1183\/13993003.02449-2016","article-title":"Randomised placebo-controlled safety and tolerability trial of FK506 (tacrolimus) for pulmonary arterial hypertension","volume":"50","author":"Spiekerkoetter","year":"2017","journal-title":"Eur Respir J"},{"key":"10.1016\/j.revmed.2023.10.450_bib1050","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1164\/rccm.202009-3481OC","article-title":"Safety and efficacy of B-cell depletion with rituximab for the treatment of systemic sclerosis\u2013associated pulmonary arterial hypertension: a multicenter, double-blind, randomized, placebo-controlled trial","volume":"204","author":"Zamanian","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.revmed.2023.10.450_bib1055","doi-asserted-by":"crossref","first-page":"2002463","DOI":"10.1183\/13993003.02463-2020","article-title":"Mendelian randomisation and experimental medicine approaches to interleukin-6 as a drug target in pulmonary arterial hypertension","volume":"59","author":"Toshner","year":"2022","journal-title":"Eur Respir J"},{"key":"10.1016\/j.revmed.2023.10.450_bib1060","doi-asserted-by":"crossref","first-page":"1478","DOI":"10.1056\/NEJMoa2213558","article-title":"Phase 3 trial of sotatercept for treatment of pulmonary arterial hypertension","volume":"388","author":"Hoeper","year":"2023","journal-title":"N Engl J Med"},{"key":"10.1016\/j.revmed.2023.10.450_bib1065","doi-asserted-by":"crossref","first-page":"1884","DOI":"10.1016\/S0140-6736(22)01601-4","article-title":"The evolving landscape of pulmonary arterial hypertension clinical trials","volume":"400","author":"Weatherald","year":"2022","journal-title":"Lancet"},{"key":"10.1016\/j.revmed.2023.10.450_bib1070","doi-asserted-by":"crossref","first-page":"2456","DOI":"10.1002\/art.30423","article-title":"Systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease: impact of pulmonary arterial hypertension therapies","volume":"63","author":"Le Pavec","year":"2011","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.revmed.2023.10.450_bib1075","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1056\/NEJMoa2008470","article-title":"Inhaled treprostinil in pulmonary hypertension due to interstitial lung disease","volume":"384","author":"Waxman","year":"2021","journal-title":"N Engl J Med"},{"key":"10.1016\/j.revmed.2023.10.450_bib1080","doi-asserted-by":"crossref","first-page":"e345","DOI":"10.1016\/j.lpm.2014.01.020","article-title":"Lung and heart-lung transplantation for systemic sclerosis patients. A monocentric experience of 13 patients, review of the literature and position paper of a multidisciplinary Working Group","volume":"43","author":"Launay","year":"2014","journal-title":"Presse Med"},{"key":"10.1016\/j.revmed.2023.10.450_bib1085","doi-asserted-by":"crossref","first-page":"910","DOI":"10.1016\/j.jtcvs.2011.08.055","article-title":"Outcome of patients with pulmonary arterial hypertension referred for lung transplantation: a 14-year single-center experience","volume":"143","author":"de Perrot","year":"2012","journal-title":"J Thorac Cardiovasc Surg"},{"key":"10.1016\/j.revmed.2023.10.450_bib1090","doi-asserted-by":"crossref","first-page":"903","DOI":"10.1016\/j.healun.2018.03.003","article-title":"Lung transplantation for scleroderma lung disease: an international, multicenter, observational cohort study","volume":"37","author":"Prad\u00e8re","year":"2018","journal-title":"J Heart Lung Transplant"}],"container-title":["La Revue de M\u00e9decine Interne"],"original-title":[],"language":"fr","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0248866323012122?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0248866323012122?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T16:47:00Z","timestamp":1759337220000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0248866323012122"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,1]]},"references-count":109,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2024,1]]}},"alternative-id":["S0248866323012122"],"URL":"https:\/\/doi.org\/10.1016\/j.revmed.2023.10.450","relation":{},"ISSN":["0248-8663"],"issn-type":[{"value":"0248-8663","type":"print"}],"subject":[],"published":{"date-parts":[[2024,1]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Pulmonary hypertension in connective tissue diseases: What every CTD specialist should know\u00a0\u2013\u00a0but is afraid to ask!","name":"articletitle","label":"Article Title"},{"value":"La Revue de M\u00e9decine Interne","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.revmed.2023.10.450","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2023 Soci\u00e9t\u00e9 Nationale Fran\u00e7aise de M\u00e9decine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.","name":"copyright","label":"Copyright"}]}}