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Circ Res. 2014 Jun 20;115(1):165-75. doi: 10.1161/CIRCRESAHA.113.301141.

Inflammation and immunity in the pathogenesis of pulmonary arterial hypertension.

Author information

1
From the Cardiovascular Institute and Department of Pediatrics (M.R.) and Department of Medicine (M.R.N.), Stanford University School of Medicine, CA; INSERM UMR_S 999, LabEx LERMIT, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson and Université Paris-Sud, School of Medicine, Le Kremlin-Bicêtre (C.G., M.H.); and AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital de Bicêtre, France (M.H.). marlener@stanford.edu.
2
From the Cardiovascular Institute and Department of Pediatrics (M.R.) and Department of Medicine (M.R.N.), Stanford University School of Medicine, CA; INSERM UMR_S 999, LabEx LERMIT, Centre Chirurgical Marie Lannelongue, Le Plessis-Robinson and Université Paris-Sud, School of Medicine, Le Kremlin-Bicêtre (C.G., M.H.); and AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital de Bicêtre, France (M.H.).

Abstract

This review summarizes an expanding body of knowledge indicating that failure to resolve inflammation and altered immune processes underlie the development of pulmonary arterial hypertension. The chemokines and cytokines implicated in pulmonary arterial hypertension that could form a biomarker platform are discussed. Pre-clinical studies that provide the basis for dysregulated immunity in animal models of the disease are reviewed. In addition, we present therapies that target inflammatory/immune mechanisms that are currently enrolling patients, and discuss others in development. We show how genetic and metabolic abnormalities are inextricably linked to dysregulated immunity and adverse remodeling in the pulmonary arteries.

KEYWORDS:

hypertension, pulmonary; leukotriene B4

PMID:
24951765
PMCID:
PMC4097142
DOI:
10.1161/CIRCRESAHA.113.301141
[Indexed for MEDLINE]
Free PMC Article

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