Research advances in connective tissue disease-associated pulmonary arterial hypertension with an emphasis on the Chinese population.

Baocheng Liu, Zihao Wang, Xin Li, Qiyao Liu, Jingyu Zhu

Abstract readReview

In one paragraph

Review in Frontiers in medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed

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1 · What the graph read from it

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4 · The record

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5 · Who and what money

Authors and funding

5 authors.

Baocheng Liu *Heze Traditional Chinese Medicine Hospital Affiliated with Shandong University of Traditional Chinese Medicine, Heze, Shandong, China.

Zihao Wang *Department of Gastroenterology, Jinan Central Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China.

Xin LiDepartment of Gastroenterology, Jinan Central Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China.

Qiyao LiuHeze Traditional Chinese Medicine Hospital Affiliated with Shandong University of Traditional Chinese Medicine, Heze, Shandong, China.

Jingyu ZhuDepartment of Gastroenterology, Jinan Central Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

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 Sjögren's 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.

Indexed as

clinical featuresconnective tissue diseaseHan Chinese populationsprognosis and treatmentpulmonary arterial hypertension

Identifiers

PMID42158142

DOI10.3389/fmed.2026.1811378

PMCPMC13180610

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