Abstract: Schistosomiasis⁃associated pulmonary arterial hypertension (Sch⁃PAH) is categorized as WHO Group I PAH because its clinical manifestations, laboratory and hemodynamic features share with PAH of other etiologies, such as idiopathic, heritable, HIV and autoimmune disorders. Sch⁃PAH is usually a life⁃threatening complication of hepatosplenic schistosomiasis characterized by changes in the vascular wall, remodeling and vasoconstriction with lesions primarily located in the precapillary segments of the pulmonary vasculature, which may result in a marked and sustained increase in pulmonary vascular resistance, right ventricular failure and ultimately death. Although egg deposition into lung and subsequent inflammatory cascades are key factors in the pathogenesis of Sch⁃PAH, the exact pathogenesis, course of disease and treatment of Sch⁃PAH remain largely uncertain. This review mainly discusses the pathophysiological and immunological mechanisms of Sch⁃PAH, so as to provide insights into the clinical diagnosis and treatment of Sch⁃PAH.

Key words: Schistosomiasis, Pulmonary arterial hypertension, Pathogenesis, Vascular remodeling, Diagnosis, Treatment

摘要： 血吸虫病相关性肺动脉高压因其临床表现、实验室检查和血流动力学特征与特发性、遗传性、HIV和自身免疫性疾病所致肺动脉高压类似而被归为WHO 定义的第I类肺动脉高压，通常是肝脾型血吸虫病的一种危及生命的并发症。以血管壁改变、重构、血管收缩为特征，病变主要位于肺血管的毛细血管前段，可导致肺血管阻力显著和持续增加、右心室衰竭乃至死亡。尽管虫卵沉积到肺部和随后的炎症级联反应是血吸虫病相关性肺动脉高压发生的关键因素，但其确切发病机制、病程和治疗仍有很大不确定性。本文主要探讨血吸虫病相关性肺动脉高压的病理生理学和免疫学机制，以期为临床诊断和治疗提供帮助。 　

关键词: 血吸虫病, 肺动脉高压, 发病机制, 血管重构, 诊断, 治疗