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Increased Antielastase Activity in Idiopathic Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension.

Accepted version
Peer-reviewed

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Type

Article

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Authors

Lodge, Katharine 
Newnham, Michael 
Bunclark, Katherine 
Toshner, Mark 

Abstract

Pulmonary arterial hypertension (PAH) is characterized by abnormal remodeling and occlusion of pre-capillary arterioles in the lung with a subsequent increase in pulmonary vascular resistance. This can lead to right ventricular hypertrophy and ultimately right heart failure. Elastase is implicated in the pathobiology of PAH with evidence including: ultra-structural studies showing increased elastase activity in pulmonary arteries from children with congenital heart disease-associated PAH (1), increased elastase release from peripheral blood neutrophils isolated from patients with pulmonary hypertension compared with healthy controls (2) and elevated plasma concentrations of elastase in patients with idiopathic PAH (IPAH) (3).

Description

Keywords

Biomarkers, Case-Control Studies, Chronic Disease, Familial Primary Pulmonary Hypertension, Female, Humans, Leukocyte Elastase, Male, Middle Aged, Pulmonary Artery, Thromboembolism, alpha 1-Antitrypsin

Journal Title

Am J Respir Cell Mol Biol

Conference Name

Journal ISSN

1044-1549
1535-4989

Volume Title

59

Publisher

American Thoracic Society
Sponsorship
British Heart Foundation (None)
British Heart Foundation (PG/15/39/31519)
British Heart Foundation (PG/17/1/32532)
British Heart Foundation (PG/17/58/33134)
British Heart Foundation (None)