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.