{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,2]],"date-time":"2025-07-02T04:19:00Z","timestamp":1751429940274,"version":"3.41.0"},"reference-count":27,"publisher":"Walter de Gruyter GmbH","issue":"2","license":[{"start":{"date-parts":[[2025,6,1]],"date-time":"2025-06-01T00:00:00Z","timestamp":1748736000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,7,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec id=\"j_rir-2025-0012_s_101\">\n                  <jats:title>Background and Objectives<\/jats:title>\n                  <jats:p>Pulmonary arterial hypertension (PAH) is a life-threatening condition that requires optimized medical therapy to maintain a low-risk profile. This study assessed the effects of initial PAH-specific combination therapy with tadalafil\/sildenafil on clinical and functional outcomes in a real-world setting.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_rir-2025-0012_s_102\">\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We conducted a multicenter retrospective study of 85 patients diagnosed with connective tissue disease-associated PAH (CTD-PAH) <jats:italic>via<\/jats:italic> right heart catheterization from 2009 to 2023. Data on treatment regimens and efficacy measures, including 6-min walk distance (6MWD), N-terminal pro-B-type natriuretic peptide (NT-pro BNP), soluble suppression of tumorigenicity 2 (sST2), World Health Organization (WHO) functional class, risk stratification, treat-to-target status and survival, were collected.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_rir-2025-0012_s_103\">\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Patients receiving initial combination therapy with endothelin receptor antagonists (ERAs) and phosphodiesterase-5 inhibitors showed varied improvements. The tadalafil plus ERAs combination significantly reduced NT-pro BNP levels and improved risk status (<jats:italic>P<\/jats:italic> &lt; 0.05). Notable enhancements in 6MWD, soluble ST2, and WHO functional class were observed in the tadalafil plus ERA group (<jats:italic>P<\/jats:italic> &lt; 0.001), but not in the sildenafil group (<jats:italic>P<\/jats:italic> &gt; 0.05). Additionally, 1-year treat-to-target rates were higher in the tadalafil plus ERA group (73.5%) than in the sildenafil group (45.6%, <jats:italic>P<\/jats:italic> = 0.005).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_rir-2025-0012_s_104\">\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>These findings suggest that tadalafil combined with ERAs leads to better improvements in exercise capacity, functional class, and treatment goals compared to sildenafil-based regimens, offering valuable insights for optimizing CTD-PAH treatment.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1515\/rir-2025-0012","type":"journal-article","created":{"date-parts":[[2025,7,1]],"date-time":"2025-07-01T17:55:18Z","timestamp":1751392518000},"page":"90-98","source":"Crossref","is-referenced-by-count":0,"title":["Tadalafil plus endothelin receptor antagonists in connective tissue disease-associated pulmonary arterial hypertension: A multicenter study on exercise capacity and cardiac outcomes"],"prefix":"10.1515","volume":"6","author":[{"given":"Qianwen","family":"Wu","sequence":"first","affiliation":[{"name":"Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University , Nanjing , Jiangsu Province , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hua","family":"Ma","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, The Affiliated Hospital of Xuzhou Medical University , Xuzhou , Jiangsu Province , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dongyu","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University , Nanjing , Jiangsu Province , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Huangshu","family":"Ye","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University , Nanjing , Jiangsu Province , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhangdi","family":"Zhou","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University , Nanjing , Jiangsu Province , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ning","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, The Affiliated Hospital of Yangzhou University , Yangzhou , Jiangsu Province , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yinsu","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Radiology, The Affiliated Cancer Hospital of Nanjing Medical University , Nanjing , Jiangsu Province , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ting","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Wuxi People\u2019s Hospital , Wuxi , Jiangsu Province , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaoxuan","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University , Nanjing , Jiangsu Province , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miaojia","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University , Nanjing , Jiangsu Province , China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3079-6383","authenticated-orcid":false,"given":"Qiang","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University , Nanjing , Jiangsu Province , China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"374","published-online":{"date-parts":[[2025,7,1]]},"reference":[{"key":"2025070117550982482_j_rir-2025-0012_ref_001","doi-asserted-by":"crossref","unstructured":"Khanna D, Zhao C, Saggar R, et al. 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