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Schistosomiasis-Associated Pulmonary Arterial Hypertension

  • Chapter
Diagnosis and Management of Pulmonary Hypertension

Part of the book series: Respiratory Medicine ((RM,volume 12))

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Abstract

Schistosomiasis is the third most common endemic disease and has been considered the most common cause of pulmonary arterial hypertension (PAH) in the world. Around 6 % of people with chronic schistosomiasis in endemic areas are affected. Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) is classified by the World Health Organization as group 1 pulmonary arterial hypertension because it has clinical, pathological, and hemodynamic characteristic that are similar to idiopathic pulmonary arterial hypertension. Sch-PAH is a severe disease that affects mainly middle-aged women. Symptoms and signs of right-heart insufficiency may be present, such as dyspnea, syncope, and peripheral edema. It has been demonstrated that Sch-PAH has a more benign clinical course and a better survival than idiopathic pulmonary hypertension. There are few reports of any beneficial effect from specific therapy directed toward group 1 PAH in this population of patients. The mechanisms responsible for the development of pulmonary hypertension in patients with schistosomiasis are unknown. There is growing evidence that vessel obstruction and granulomatous reaction to pulmonary embolization of eggs is not the sole mechanism that causes the disease. Further studies are needed to better elucidate the pathogenesis of Sch-PAH and to develop targeted therapies for this devastating disease.

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Abbreviations

6MWT:

6-min walk test

CT:

Computed tomography

HIV:

Human immunodeficiency virus

IL:

Interleukin

INF γ:

Interferon γ

mPAP:

Mean pulmonary artery pressure

PAH:

Pulmonary arterial hypertension

PAP:

Pulmonary artery pressure

PAOP:

Pulmonary artery occlusion pressure

PASP:

Pulmonary artery systolic pressure

PH:

Pulmonary hypertension

PVR:

Pulmonary vascular resistance

PWP:

Pulmonary wedge pressure

Sch-PAH:

Schistosomiasis-associated pulmonary arterial hypertension

TGF β:

Transforming growth factor β

Th cells:

T helper cells

TNF α:

Tumuor necrosis factor α

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Authors and Affiliations

  1. Department of Pneumology, Hospital das Clínicas - Universidade Federal de Pernambuco, Av Prof Moraes Rego 1235, Cidade Universitária, Recife, PE, 50670-901, Brazil

    Rita de Cassia dos Santos Ferreira Ph.D.

  2. University of Pernambuco, Recife, Brazil

    Angela Pontes Bandeira M.D.

  3. Health Sciences Centre, Federal University of Pernambuco, Recife, Brazil

    Ana Lúcia Coutinho Domingues Ph.D.

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  1. Rita de Cassia dos Santos Ferreira Ph.D.
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  2. Angela Pontes Bandeira M.D.
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  3. Ana Lúcia Coutinho Domingues Ph.D.
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Correspondence to Rita de Cassia dos Santos Ferreira Ph.D. .

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Editors and Affiliations

  1. Division of Pulmonary, Sleep and Critical Care Medicine, Rhode Island Hospital, Alpert Medical School of Brown University, Providence, Rhode Island, USA

    James R. Klinger

  2. Department of Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota, USA

    Robert P. Frantz

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de Cassia dos Santos Ferreira, R., Bandeira, A.P., Domingues, A.L.C. (2015). Schistosomiasis-Associated Pulmonary Arterial Hypertension. In: Klinger, J., Frantz, R. (eds) Diagnosis and Management of Pulmonary Hypertension. Respiratory Medicine, vol 12. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-2636-7_7

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