Abstract
Schistosomiasis, especially due to Schistosoma mansoni, is a well-recognized cause of pulmonary arterial hypertension (PAH). The high prevalence of this helminthiasis makes schistosome-related PAH (Sch-PAH) one of the most common causes of this disorder worldwide. The pathogenic mechanisms underlying Sch-PAH remain largely unknown. Available evidence suggests that schistosome eggs reach the lung via portocaval shunts formed as a consequence of portal hypertension due to hepatosplenic schistosomiasis. Once deposited into the lungs, the eggs elicit an immune response resulting in periovular granuloma formation. Immune mediators drive transforming growth factor-β (TGF-β) release, which gives rise to pulmonary vascular inflammation with subsequent remodeling and development of angiomatoid and plexiform lesions. These mechanisms elicited by the eggs seem to become autonomous and the vascular lesions progress independently of the antigen. Portopulmonary hypertension, which pathogenesis is still uncertain, may also play a role in the genesis of Sch-PAH. Recently, there have been substantial advances in the diagnosis and treatment of PAH, but it remains a difficult condition to recognize and manage, and patients still die prematurely from right-heart failure. Echocardiography is used for screening, and the formal diagnosis requires right-heart catheterization. The experience in treating Sch-PAH is largely limited to the phosphodiesterase type 5 inhibitors, with evidence suggesting that these vasodilators improve symptoms and may also improve survival. Considering the great deal of uncertainty about Sch-PAH pathogenesis, course, and treatment, the aim of this review is to summarize current knowledge on this condition emphasizing its pathogenesis.
Highlights
Pulmonary hypertension (PH) is a hemodynamic and pathophysiologic condition that comprises heterogeneous disorders, leads to right-heart failure if untreated, and carries substantial morbidity and mortality [1,2,3,4]
Even in non-endemic areas, epidemiologic information about schistosomiasis exposure is important to be elicited. This form of PH develops in patients with the severe hepatosplenic form of schistosomiasis, especially due to S. mansoni infection, following egg embolization to the lungs via collaterals that form as a consequence of portal hypertension
Sch-pulmonary arterial hypertension (PAH) pathogenesis is largely unknown, progressive remodeling of pulmonary vasculature is considered a key mechanism in the genesis of this disease
Summary
Pulmonary hypertension (PH) is a hemodynamic and pathophysiologic condition that comprises heterogeneous disorders, leads to right-heart failure if untreated, and carries substantial morbidity and mortality [1,2,3,4]. It is defined as a mean pulmonary arterial pressure exceeding 20 mmHg at rest as assessed by right-heart catheterization [5]. Over the last two decades, there have been substantial advances in the diagnosis and treatment of Sch-PAH, but it remains a difficult condition to recognize and manage, and patients still die prematurely from right-heart failure [11]. The aim of this mini-review is to summarize the current knowledge regarding Sch-PAH emphasizing its pathogenesis
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