Article
Association of Rare Variants in Pulmonary Arterial Hypertension-Related Genes With a Distinct Vasculopathy Phenotype and Worse Outcomes in Patients With Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension.
Chest - 1 May 2026
Qian Junyan, Yang Xinzhuang, Wang Qian, Zhao Jiuliang, Ma Leyao, Ding Yufang, Yuan Qizhi, Li Mucong, Liu Weida, Liu Yongtai, Tian Zhuang, Wang Yanhong, Yang Weixian, Zeng Xiaofeng, Wang Xiaojian, Li Mengtao
Abstract excerpt
BACKGROUND: Systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) exhibits marked clinical heterogeneity. Although pathogenic variants in the BMPR2 gene and other PAH-related genes drive pulmonary vascular remodeling in idiopathic or familial PAH, their role in SLE-associated PAH remains unclear. RESEARCH QUESTION: What is the prevalence of rare variants in PAH-related genes in...
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