{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T20:49:28Z","timestamp":1791146968179,"version":"4.1.0"},"reference-count":45,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2009,6,1]],"date-time":"2009-06-01T00:00:00Z","timestamp":1243814400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2009,6,1]],"date-time":"2009-06-01T00:00:00Z","timestamp":1243814400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","jhltonline.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Heart and Lung Transplantation"],"published-print":{"date-parts":[[2009,6]]},"DOI":"10.1016\/j.healun.2009.02.016","type":"journal-article","created":{"date-parts":[[2009,5,28]],"date-time":"2009-05-28T05:23:47Z","timestamp":1243488227000},"page":"621-627","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":35,"title":["Comparison of Baseline Characteristics and Survival Between Patients With Idiopathic and Connective Tissue Disease\u2013related Pulmonary Arterial Hypertension"],"prefix":"10.1016","volume":"28","author":[{"given":"Maria J.","family":"Ruiz-Cano","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pilar","family":"Escribano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rafael","family":"Alonso","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan","family":"Delgado","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Carreira","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teresa","family":"Velazquez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel A. Gomez","family":"Sanchez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"S\u00e1enz de la Calzada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"suppl 1","key":"10.1016\/j.healun.2009.02.016_bib1","doi-asserted-by":"crossref","first-page":"S5","DOI":"10.1016\/j.jacc.2004.02.037","article-title":"Clinical classification of pulmonary hypertension","volume":"43","author":"Simonneau","year":"2004","journal-title":"J Am Coll Cardiol"},{"key":"10.1016\/j.healun.2009.02.016_bib2","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1046\/j.1365-3083.2000.00733.x","article-title":"Fine specificity of anti-fibrillin-1 autoantibodies in primary pulmonary hypertension syndrome","volume":"51","author":"Morse","year":"2000","journal-title":"Scand J Immunol"},{"key":"10.1016\/j.healun.2009.02.016_bib3","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1016\/S0889-857X(05)70335-5","article-title":"Pulmonary hypertension in systemic autoimmune disease","volume":"23","author":"Gurubhagavatula","year":"1997","journal-title":"Rheum Dis Clin N Am"},{"key":"10.1016\/j.healun.2009.02.016_bib4","doi-asserted-by":"crossref","first-page":"394","DOI":"10.7326\/0003-4819-86-4-394","article-title":"Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma)","volume":"86","author":"Salerni","year":"1977","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.healun.2009.02.016_bib5","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1016\/0046-8177(90)90002-M","article-title":"The pulmonary pathologic manifestations of the CREST syndrome","volume":"21","author":"Yousem","year":"1990","journal-title":"Hum Pathol"},{"key":"10.1016\/j.healun.2009.02.016_bib6","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1002\/art.1780290409","article-title":"Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis","volume":"29","author":"Stupi","year":"1986","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.healun.2009.02.016_bib7","doi-asserted-by":"crossref","first-page":"1198","DOI":"10.1161\/01.CIR.80.5.1198","article-title":"Histopathology of primary pulmonary hypertension: a qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension Registry","volume":"80","author":"Pietra","year":"1989","journal-title":"Circulation"},{"key":"10.1016\/j.healun.2009.02.016_bib8","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1378\/chest.123.2.344","article-title":"Pulmonary arterial hypertension related to systemic sclerosis","volume":"123","author":"Kawut","year":"2003","journal-title":"Chest"},{"key":"10.1016\/j.healun.2009.02.016_bib9","doi-asserted-by":"crossref","first-page":"896","DOI":"10.1056\/NEJMoa012212","article-title":"Bosentan therapy for pulmonary arterial hypertension","volume":"346","author":"Rubin","year":"2002","journal-title":"N Engl J Med"},{"key":"10.1016\/j.healun.2009.02.016_bib10","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1093\/rheumatology\/35.10.989","article-title":"Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients","volume":"35","author":"Koh","year":"1996","journal-title":"Br J Rheumatol"},{"key":"10.1016\/j.healun.2009.02.016_bib11","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1093\/rheumatology\/40.4.453","article-title":"Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival","volume":"40","author":"MacGregor","year":"2001","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.healun.2009.02.016_bib12","doi-asserted-by":"crossref","first-page":"3043","DOI":"10.1002\/art.22069","article-title":"Clinical differences between idiopathic and scleroderma-related pulmonary hypertension","volume":"54","author":"Fisher","year":"2006","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.healun.2009.02.016_bib13","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1056\/NEJM199602013340504","article-title":"A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension","volume":"334","author":"Barst","year":"1996","journal-title":"N Engl J Med"},{"key":"10.1016\/j.healun.2009.02.016_bib14","doi-asserted-by":"crossref","first-page":"2148","DOI":"10.1056\/NEJMoa050010","article-title":"Sildenafil citrate therapy for pulmonary artery hypertension","volume":"353","author":"Gali\u00e8","year":"2005","journal-title":"N Engl J Med"},{"key":"10.1016\/j.healun.2009.02.016_bib15","doi-asserted-by":"crossref","first-page":"1441","DOI":"10.1016\/j.healun.2003.09.024","article-title":"Sildenafil for pulmonary hypertension after heart transplantation","volume":"23","author":"Kulkarni","year":"2004","journal-title":"J Heart Lung Transplant"},{"key":"10.1016\/j.healun.2009.02.016_bib16","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1016\/j.healun.2004.01.017","article-title":"Novel use of sildenafil in the treatment of portopulmonary hypertension","volume":"24","author":"Chua","year":"2005","journal-title":"J Heart Lung Transplant"},{"key":"10.1016\/j.healun.2009.02.016_bib17","doi-asserted-by":"crossref","first-page":"926","DOI":"10.1136\/hrt.2005.069484","article-title":"Systemic sclerosis associated pulmonary hypertension: improved survival in the current era","volume":"92","author":"Williams","year":"2006","journal-title":"Heart"},{"key":"10.1016\/j.healun.2009.02.016_bib18","doi-asserted-by":"crossref","first-page":"216","DOI":"10.7326\/0003-4819-107-2-216","article-title":"Primary pulmonary hypertension: a national prospective study","volume":"107","author":"Rich","year":"1987","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.healun.2009.02.016_bib19","series-title":"World symposium on primary pulmonary hypertension","article-title":"Executive summary from the World Symposium on Primary Pulmonary Hypertension 1998","year":"1998"},{"key":"10.1016\/j.healun.2009.02.016_bib20","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"},{"issue":"suppl 1","key":"10.1016\/j.healun.2009.02.016_bib21","doi-asserted-by":"crossref","first-page":"35S","DOI":"10.1378\/chest.126.1_suppl.35S","article-title":"Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines","volume":"126","author":"Badesch","year":"2004","journal-title":"Chest"},{"key":"10.1016\/j.healun.2009.02.016_bib22","doi-asserted-by":"crossref","first-page":"2243","DOI":"10.1016\/j.ehj.2004.09.014","article-title":"Guidelines on diagnosis and treatment of pulmonary arterial hypertension","volume":"25","author":"Galie","year":"2004","journal-title":"Eur Heart J"},{"key":"10.1016\/j.healun.2009.02.016_bib23","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1056\/NEJM199801293380501","article-title":"Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension","volume":"338","author":"Laughlin","year":"1998","journal-title":"N Engl J Med"},{"key":"10.1016\/j.healun.2009.02.016_bib24","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","volume":"132","author":"Badesch","year":"2000","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.healun.2009.02.016_bib25","first-page":"703","article-title":"International guidelines for the selection of lung transplant candidates","volume":"17","author":"Maurer","year":"1998","journal-title":"J Heart Lung Transplant"},{"issue":"suppl","key":"10.1016\/j.healun.2009.02.016_bib26","doi-asserted-by":"crossref","first-page":"62S","DOI":"10.1016\/j.jacc.2004.02.042","article-title":"Endothelin receptor antagonists in pulmonary arterial hypertension","volume":"43","author":"Channick","year":"2004","journal-title":"J Am Coll Cardiol"},{"key":"10.1016\/j.healun.2009.02.016_bib27","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/j.healun.2006.11.009","article-title":"Quality of life in pulmonary arterial hypertension: improvement and maintenance with bosentan","volume":"26","author":"Keogh","year":"2007","journal-title":"J Heart Lung Transplant"},{"key":"10.1016\/j.healun.2009.02.016_bib28","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1164\/ajrccm.165.6.2106079","article-title":"Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind randomized controlled trial","volume":"165","author":"Simonneau","year":"2002","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.healun.2009.02.016_bib29","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1056\/NEJMoa020204","article-title":"Inhaled iloprost in severe pulmonary hypertension","volume":"347","author":"Olschewski","year":"2002","journal-title":"N Engl J Med"},{"key":"10.1016\/j.healun.2009.02.016_bib30","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1016\/S0735-1097(02)02012-0","article-title":"Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival","volume":"4","author":"Sitbon","year":"2002","journal-title":"J Am Coll Cardiol"},{"key":"10.1016\/j.healun.2009.02.016_bib31","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.healun.2009.02.016_bib32","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1016\/S0140-6736(98)02111-4","article-title":"Primary pulmonary hypertension","volume":"352","author":"Gaine","year":"1998","journal-title":"Lancet"},{"key":"10.1016\/j.healun.2009.02.016_bib33","doi-asserted-by":"crossref","first-page":"1214","DOI":"10.1016\/S0735-1097(02)01744-8","article-title":"Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension","volume":"39","author":"Raymond","year":"2002","journal-title":"J Am Coll Cardiol"},{"key":"10.1016\/j.healun.2009.02.016_bib34","doi-asserted-by":"crossref","first-page":"2098","DOI":"10.1378\/chest.124.6.2098","article-title":"Age and risk of pulmonary arterial hypertension in scleroderma","volume":"124","author":"Schachna","year":"2003","journal-title":"Chest"},{"key":"10.1016\/j.healun.2009.02.016_bib35","doi-asserted-by":"crossref","first-page":"1088","DOI":"10.1136\/ard.62.11.1088","article-title":"Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach","volume":"62","author":"Mukerjee","year":"2003","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.healun.2009.02.016_bib36","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1093\/rheumatology\/36.2.239","article-title":"Comparison of Doppler echocardiography and right heart catheterisation to assess pulmonary hypertension in systemic sclerosis","volume":"36","author":"Denton","year":"1997","journal-title":"Br J Rheumatol"},{"key":"10.1016\/j.healun.2009.02.016_bib37","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1136\/thx.54.3.273","article-title":"Treatment of pulmonary hypertension secondary to connective tissue diseases","volume":"54","author":"Sanchez","year":"1999","journal-title":"Thorax"},{"key":"10.1016\/j.healun.2009.02.016_bib38","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1177\/096120339300200606","article-title":"Pulmonary arterial hypertension responsive to immunosuppressive treatment in SLE","volume":"2","author":"Morelli","year":"1993","journal-title":"Lupus"},{"key":"10.1016\/j.healun.2009.02.016_bib39","doi-asserted-by":"crossref","first-page":"1687","DOI":"10.1016\/0002-9149(91)90330-N","article-title":"Acute and long term effects of nifedipine on pulmonary and systemic haemodynamics in patients with pulmonary hypertension associated with diffuse systemic sclerosis, the CREST syndrome and mixed connective tissue disease","volume":"68","author":"Alpert","year":"1991","journal-title":"Am J Cardiol"},{"key":"10.1016\/j.healun.2009.02.016_bib40","doi-asserted-by":"crossref","first-page":"343","DOI":"10.7326\/0003-4819-115-5-343","article-title":"Survival in patients with primary pulmonary hypertension: results from a national prospective registry","volume":"115","author":"D'Alonzo","year":"1991","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.healun.2009.02.016_bib41","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1002\/art.21538","article-title":"Pulmonary arterial hypertension is a major mortality factor in diffuse systemic sclerosis, independent of interstitial lung disease","volume":"54","author":"Trad","year":"2006","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.healun.2009.02.016_bib42","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":"Dorfmuller","year":"2007","journal-title":"Hum Pathol"},{"key":"10.1016\/j.healun.2009.02.016_bib43","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1016\/S1053-2498(03)00182-7","article-title":"Twentieth official adult lung and heart\u2013lung transplant report\u20142003","volume":"22","author":"Trulock","year":"2003","journal-title":"J Heart Lung Transplant"},{"key":"10.1016\/j.healun.2009.02.016_bib44","doi-asserted-by":"crossref","first-page":"3954","DOI":"10.1002\/art.22264","article-title":"Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension","volume":"54","author":"Schachna","year":"2006","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.healun.2009.02.016_bib45","doi-asserted-by":"crossref","first-page":"956","DOI":"10.1016\/j.healun.2005.05.019","article-title":"Registry of the International Society for Heart and Lung Transplantation: twenty-second official adult lung and heart\u2013lung transplant report\u20142005","volume":"24","author":"Trulock","year":"2005","journal-title":"J Heart Lung Transplant"}],"container-title":["The Journal of Heart and Lung Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1053249809002198?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1053249809002198?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T08:19:20Z","timestamp":1760948360000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1053249809002198"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,6]]},"references-count":45,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2009,6]]}},"alternative-id":["S1053249809002198"],"URL":"https:\/\/doi.org\/10.1016\/j.healun.2009.02.016","relation":{},"ISSN":["1053-2498"],"issn-type":[{"value":"1053-2498","type":"print"}],"subject":[],"published":{"date-parts":[[2009,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Comparison of Baseline Characteristics and Survival Between Patients With Idiopathic and Connective Tissue Disease\u2013related Pulmonary Arterial Hypertension","name":"articletitle","label":"Article Title"},{"value":"The Journal of Heart and Lung Transplantation","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.healun.2009.02.016","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2009 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}