Schistosomiasis-associated pulmonary arterial hypertension: survival in endemic area in Brazil

Abstract

HighlightsThe PHC registry equation accurately predicted survival in Sch-PAH patients.Sch-PAH survival cannot be safely predicted using only validated clinical variables only.Survival studies in endemic regions of Schistosomiasis are needed to establish the real prognosis of Sch-PAH in the world.

Schistosomiasis Pulmonary Arterial Hypertension

“…Clinical studies in SchPAH are scarce and restricted to a few PH centers in the world, largely in Brazil (67)(68)(69)(70)(71)(72)(73)(74)(75)(76). Additionally, most of the clinical data reported to date were generated in centers situated in non-endemic areas for schistosomiasis (62,64,65,(67)(68)(69).The classic comparator disease for SchPAH is IPAH, which occurs worldwide and has been much more widely studied despite a much lower disease burden (the incidence is approximately 1 case/million individuals/year) (77).…”

“…The SchPAH mortality rate has been reported at 9.7 deaths per 1000 patients/month in endemic areas ( 75 ). The clinical course of SchPAH is believed to be more benign compared to IPAH or PAH associated with connective tissue diseases ( 73 ), even in the absence of specific PAH treatment ( 69 ).…”

Schistosomiasis-Associated Pulmonary Arterial Hypertension

“…The clinical course of SchPAH may be more benign than IPAH or PH associated with connective tissue diseases, 25 even in the absence of specific PAH treatment. 26 Treatment-naïve SchPAH patients have been reported to have overall survival rates at 1, 2, and 3 years of 95%, 95%, and 86%, respectively, 26 although a more recent series from an endemic setting reported 1-, 3-, and 5-year survival estimates of 92%, 75%, and 51%, respectively, 27 figures closer to the prognosis in IPAH. A recent systematic review that included 18 studies concluded that SchPAH has a better prognosis than IPAH, 28 despite presenting with initially similar hemodynamics, although it should be cautioned that many of these patients were studied in nonendemic settings.…”

Avaliação do comportamento cronotrópico e ventilatório de pacientes com hipertensão arterial pulmonar

“…De acordo com o presente estudo, contudo, tal diferença não pode ser explicada por peculiaridades no comportamento dinâmico ao esforço, ou por alterações no balanço autonômico avaliado pela microneurografia e pela variabilidade de frequência cardíaca. A caracterização hemodinâmica dos pacientes foi igualmente semelhante a descrições prévias, dentro ou fora de locais onde a esquistossomose é endêmica 8,68 .…”

“…Embora tal achado possa ser decorrente de evolução adaptativa de longa data, à semelhança de pacientes com cardiopatia congênita, é preciso lembrar que mecanismos inflamatórios decorrentes da infecção pelo Schistosoma mansoni são importantes da gênese da HAP-Sch 17,22,23 . Também sugerindo um curso prolongado de doença, encontra-se o dado de que esses pacientes se apresentam mais velhos ao diagnóstico quando comparados aos pacientes com HAPI, como encontrado em nosso estudo e já relatado anteriormente 8,68 . Por outro lado, é possível que as alterações endoteliais nesse grupo de pacientes sejam decorrentes de diferentes mecanismos inflamatórios, ou que particularidades fisiológicas determinem evoluções clínicas distintas.…”