Demographic and clinical characteristics of pulmonary arterial hypertension caused by schistosomiasis are indistinguishable from other etiologies

Mendes, Adriano A.; Roncal, Carlos Guilhermo Piscoya; Oliveira, Flávio Roberto Azevedo de; Albuquerque, Eugenio Soares de; Góes, Gustavo; Piscoya, Isabelle Cecília de Vasconcellos; Filho, Dário Celestino Sobral

doi:10.1590/0037-8682-0418-2019

Cited by 5 publications

(5 citation statements)

References 37 publications

(39 reference statements)

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“…However, it is important to note that most of the studies included in the aforementioned systematic review were derived from a single center, raising concerns that patient pools may overlap, potentially influencing the systematic review findings. In this context, recent data from an endemic area suggest SchPAH might have a similar hemodynamic profile at diagnosis compared to other PH etiologies ( 76 ).…”

Section: Differences In Presentation and Prognosis Of Schpah Comparedmentioning

confidence: 97%

“…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).…”

Section: Differences In Presentation and Prognosis Of Schpah Comparedmentioning

confidence: 99%

“…SchPAH occurs at an approximately 1:2 male-to-female ratio, in individuals typically between 30 and 60 years old at the time of PAH diagnosis ( 67 – 76 ). SchPAH patients have a similar clinical presentation compared to IPAH such as severity of disease [as measured by New York Heart Association functional class at the time of diagnosis ( 67 , 68 )] and duration of dyspnea symptoms preceding the PAH diagnosis ( 67 ).…”

Section: Differences In Presentation and Prognosis Of Schpah Comparedmentioning

confidence: 99%

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Schistosomiasis Pulmonary Arterial Hypertension

et al. 2020

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Pulmonary arterial hypertension (PAH) is a disease of the lung blood vessels that results in right heart failure. PAH is thought to occur in about 5% to 10% of patients with hepatosplenic schistosomiasis, particularly due to S. mansoni. The lung blood vessel injury may result from a combination of embolization of eggs through portocaval shunts into the lungs causing localized Type 2 inflammatory response and vessel remodeling, triggering of autonomous pathology that becomes independent of the antigen, and high cardiac output as seen in portopulmonary hypertension. The condition is likely underdiagnosed as there is little systematic screening, and risk factors for developing PAH are not known. Screening is done by echocardiography, and formal diagnosis requires invasive right heart catheterization. Patients with Schistosoma-associated PAH show reduced functional capacity and can be treated with pulmonary vasodilators, which improves symptoms and may improve survival. There are animal models of this disease that might help in understanding disease pathogenesis and identify novel targets to screen and treatment. Pathogenic mechanisms include Type 2 immunity and activation and signaling in the TGF-β pathway. There are still major uncertainties regarding Schistosoma-associated PAH development, course and treatment.

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Section: Differences In Presentation and Prognosis Of Schpah Comparedmentioning

confidence: 97%

Section: Differences In Presentation and Prognosis Of Schpah Comparedmentioning

confidence: 99%

Section: Differences In Presentation and Prognosis Of Schpah Comparedmentioning

confidence: 99%

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Schistosomiasis Pulmonary Arterial Hypertension

et al. 2020

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“…Marked sexual dimorphism is observed in patients with pulmonary arterial hypertension with the major disease registries reporting female to male ratios that are typically greater than 2:1 and range up to 4:1 [ 3 ]. The female preponderance is observed in many different subtypes of PAH including heritable PAH, idiopathic PAH and PAH associated with specific diseases such as scleroderma, congenital heart disease, haemolytic disease, schistosomiasis and anorexigens [ 4 , 5 , 6 , 7 , 8 , 9 , 10 ].…”

Section: Introductionmentioning

confidence: 99%

Sex Dimorphism in Pulmonary Hypertension: The Role of the Sex Chromosomes

Kostyunina

McLoughlin

2021

Antioxidants

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Pulmonary hypertension (PH) is a condition characterised by an abnormal elevation of pulmonary artery pressure caused by an increased pulmonary vascular resistance, frequently leading to right ventricular failure and reduced survival. Marked sexual dimorphism is observed in patients with pulmonary arterial hypertension, a form of pulmonary hypertension with a particularly severe clinical course. The incidence in females is 2–4 times greater than in males, although the disease is less severe in females. We review the contribution of the sex chromosomes to this sex dimorphism highlighting the impact of proteins, microRNAs and long non-coding RNAs encoded on the X and Y chromosomes. These genes are centrally involved in the cellular pathways that cause increased pulmonary vascular resistance including the production of reactive oxygen species, altered metabolism, apoptosis, inflammation, vasoconstriction and vascular remodelling. The interaction with genetic mutations on autosomal genes that cause heritable pulmonary arterial hypertension such as bone morphogenetic protein 2 (BMPR2) are examined. The mechanisms that can lead to differences in the expression of genes located on the X chromosomes between females and males are also reviewed. A better understanding of the mechanisms of sex dimorphism in this disease will contribute to the development of more effective therapies for both women and men.

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“…Patients with Sch-PAH present clinical, laboratory, and hemodynamic profiles similar to those observed in PAH due to other etiologies (8). However, the pathogenic mechanisms underlying Sch-PAH remain largely unknown (9,10), though available evidence suggests that egg deposition into the lung and consequent inflammatory response are key events in the genesis of these disorder.…”

Section: Introductionmentioning

confidence: 92%

Schistosome-Associated Pulmonary Arterial Hypertension: A Review Emphasizing Pathogenesis

Ferrari

Albricker

Gonçalves

et al. 2021

Front. Cardiovasc. Med.

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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.

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Demographic and clinical characteristics of pulmonary arterial hypertension caused by schistosomiasis are indistinguishable from other etiologies

Cited by 5 publications

References 37 publications

Schistosomiasis Pulmonary Arterial Hypertension

Schistosomiasis Pulmonary Arterial Hypertension

Sex Dimorphism in Pulmonary Hypertension: The Role of the Sex Chromosomes

Schistosome-Associated Pulmonary Arterial Hypertension: A Review Emphasizing Pathogenesis

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