Chest
Rare Variants in Pulmonary Arterial Hypertension-Related Genes Associate With a Distinct Vasculopathy Phenotype and Worse Outcomes in Patients With Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension.
Junyan Qian, Xinzhuang Yang, Qian Wang, Jiuliang Zhao, Leyao Ma, Yufang Ding, Qizhi Yuan, Mucong Li, Weida Liu, Yongtai Liu, Zhuang Tian, Yanhong Wang, Weixian Yang, Xiaofeng Zeng, Xiaojian Wang
Published: 202510.1016/j.chest.2025.11.038
Abstract
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…
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