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