Schistosomiasis-Associated Pulmonary Arterial Hypertension

Abstract

Schistosomiasis is a major cause of group 1 pulmonary arterial hypertension (PAH) worldwide. Schistosomiasis results from a parasitic infection present in over 200 million individuals worldwide. Schistosomiasis-associated PAH was initially thought to be obstructive due to egg embolization but has a pulmonary vascular pathology like other forms of group 1 PAH and can be treated using conventional PAH therapies. Mechanisms that underlie the development of schistosomiasis-associated PAH include type 2 inflammation which triggers TGF-β signaling; importantly, TGF-β signaling is a pathway shared with other PAH etiologies. However, many things which are unknown about this disease remain, including if the lung vascular pathology results from egg embolization causing localized inflammation and vessel remodeling, or if this is a form of portopulmonary hypertension resulting from schistosomiasis liver disease.