{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,5]],"date-time":"2025-07-05T04:09:58Z","timestamp":1751688598077,"version":"3.37.3"},"reference-count":96,"publisher":"F1000 Research Ltd","license":[{"start":{"date-parts":[[2019,12,19]],"date-time":"2019-12-19T00:00:00Z","timestamp":1576713600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["f1000research.com"],"crossmark-restriction":false},"short-container-title":["F1000Res"],"abstract":"<ns4:p>Pulmonary arterial hypertension, categorized as group 1 pulmonary hypertension by the World Health Organization classification system, represents a major complication of systemic sclerosis resulting from pulmonary vascular involvement of the disease. The high mortality seen in systemic sclerosis-associated pulmonary arterial hypertension is likely due to the impairment of right ventricular systolic function and the coexistence of other non-group-1 pulmonary hypertension phenotypes that may negatively impact clinical response to pulmonary arterial hypertension-targeted therapy. This review highlights two areas of recent advances regarding the management of systemic sclerosis patients with pulmonary hypertension: the tolerability of pulmonary arterial hypertension-targeted therapy in the presence of mild to moderate interstitial lung disease and the potential clinical significance of the antifibrotic effect of soluble guanylate cyclase stimulators demonstrated in preclinical studies.<\/ns4:p>","DOI":"10.12688\/f1000research.20313.1","type":"journal-article","created":{"date-parts":[[2019,12,19]],"date-time":"2019-12-19T10:06:00Z","timestamp":1576749960000},"page":"2124","update-policy":"https:\/\/doi.org\/10.12688\/f1000research.crossmark-policy","source":"Crossref","is-referenced-by-count":5,"title":["The role of pulmonary arterial hypertension-targeted therapy in systemic sclerosis"],"prefix":"10.12688","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5739-8369","authenticated-orcid":false,"given":"Michael H","family":"Lee","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Todd M","family":"Bull","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2560","published-online":{"date-parts":[[2019,12,19]]},"reference":[{"key":"ref-1","doi-asserted-by":"publisher","first-page":"152-7","DOI":"10.1016\/s1568-9972(03)00004-1","article-title":"New insights into the pathogenesis of systemic sclerosis.","volume":"2","author":"M Tamby","year":"2003","journal-title":"Autoimmun Rev."},{"key":"ref-2","first-page":"198-202","article-title":"Systemic sclerosis: An update.","volume":"66","author":"J Varga","year":"2008","journal-title":"Bull NYU Hosp Jt Dis."},{"key":"ref-3","first-page":"1573-6","article-title":"Criteria for the classification of early systemic sclerosis.","volume":"28","author":"E LeRoy","year":"2001","journal-title":"J Rheumatol."},{"key":"ref-4","doi-asserted-by":"crossref","first-page":"255-73, vi","DOI":"10.1016\/S0889-857X(03)00023-1","article-title":"Natural history of systemic sclerosis and the assessment of disease activity, severity, functional status, and psychologic well-being.","volume":"29","author":"T Medsger","year":"2003","journal-title":"Rheum Dis Clin North Am."},{"key":"ref-5","doi-asserted-by":"publisher","first-page":"444-51","DOI":"10.1002\/1529-0131(200002)43:2<444::AID-ANR27>3.0.CO;2-G","article-title":"Systemic sclerosis sine scleroderma: Demographic, clinical, and serologic features and survival in forty-eight patients.","volume":"43","author":"H Poormoghim","year":"2000","journal-title":"Arthritis Rheum."},{"key":"ref-6","doi-asserted-by":"publisher","first-page":"78-83","DOI":"10.1007\/s12016-010-8198-y","article-title":"Diagnosis and classification of systemic sclerosis.","volume":"40","author":"E Hachulla","year":"2011","journal-title":"Clin Rev Allergy Immunol."},{"key":"ref-7","doi-asserted-by":"publisher","first-page":"940-4","DOI":"10.1136\/ard.2006.066068","article-title":"Changes in causes of death in systemic sclerosis, 1972-2002.","volume":"66","author":"V Steen","year":"2007","journal-title":"Ann Rheum Dis."},{"key":"ref-8","doi-asserted-by":"publisher","first-page":"1809-15","DOI":"10.1136\/ard.2009.114264","article-title":"Causes and risk factors for death in systemic sclerosis: A study from the EULAR Scleroderma Trials and Research (EUSTAR) database.","volume":"69","author":"A Tyndall","year":"2010","journal-title":"Ann Rheum Dis."},{"key":"ref-9","doi-asserted-by":"publisher","first-page":"304-8","DOI":"10.1093\/rheumatology\/ken488","article-title":"Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French Itin\u00e9rAIR-Scl\u00e9rodermie study.","volume":"48","author":"E Hachulla","year":"2009","journal-title":"Rheumatology (Oxford)."},{"key":"ref-10","doi-asserted-by":"publisher","first-page":"151-7","DOI":"10.1164\/rccm.200806-953OC","article-title":"Connective Tissue Disease-associated Pulmonary Arterial Hypertension in the Modern Treatment Era.","volume":"179","author":"R Condliffe","year":"2009","journal-title":"Am J Respir Crit Care Med."},{"key":"ref-11","doi-asserted-by":"publisher","first-page":"1940-6","DOI":"10.1136\/annrheumdis-2012-202489","article-title":"Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era.","volume":"72","author":"D Launay","year":"2013","journal-title":"Ann Rheum Dis."},{"key":"ref-12","doi-asserted-by":"publisher","first-page":"1855-6","DOI":"10.1183\/13993003.51032-2015","article-title":"2015 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. 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