{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,5]],"date-time":"2026-05-05T02:55:54Z","timestamp":1777949754834,"version":"3.51.4"},"reference-count":157,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T00:00:00Z","timestamp":1777852800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:p>Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) is a severe pulmonary complication of connective tissue diseases (CTDs) that occurs in approximately 30% of adult patients with pulmonary arterial hypertension (PAH) and is associated with a substantial increase in mortality risk. Its incidence varies widely across regions: in Europe and North America, systemic sclerosis-associated PAH (SSc-PAH) predominates, whereas in China, systemic lupus erythematosus-associated PAH (SLE-PAH) and Sj\u00f6gren\u2019s syndrome-associated PAH (SS-PAH) are more common. However, contemporary data on clinical characteristics and prognosis in Han Chinese populations are limited, indicating a need for targeted studies to optimize diagnostic and therapeutic strategies and improve patient outcomes. This review summarizes the epidemiology, pathogenesis, clinical features, and prognostic factors of CTD-PAH and discusses potential implications for clinical management.<\/jats:p>","DOI":"10.3389\/fmed.2026.1811378","type":"journal-article","created":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T04:25:00Z","timestamp":1777868700000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Research advances in connective tissue disease-associated pulmonary arterial hypertension with an emphasis on the Chinese population"],"prefix":"10.3389","volume":"13","author":[{"given":"Baocheng","family":"Liu","sequence":"first","affiliation":[{"name":"Heze Traditional Chinese Medicine Hospital Affiliated with Shandong University of Traditional Chinese Medicine","place":["Heze, Shandong, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zihao","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Jinan Central Hospital Affiliated to Shandong First Medical University","place":["Jinan, Shandong, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xin","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Jinan Central Hospital Affiliated to Shandong First Medical University","place":["Jinan, Shandong, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Qiyao","family":"Liu","sequence":"additional","affiliation":[{"name":"Heze Traditional Chinese Medicine Hospital Affiliated with Shandong University of Traditional Chinese Medicine","place":["Heze, Shandong, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jingyu","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Jinan Central Hospital Affiliated to Shandong First Medical University","place":["Jinan, Shandong, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,5,4]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"103506","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","year":"2024","journal-title":"Autoimmun Rev"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"67","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), 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