Inflammation and Immunity in the Pathogenesis of Pulmonary Arterial Hypertension

2014/06/20 by Marlene Rabinovitch, Christophe Guignabert, Marc Humbert +1 · 29 citations
Medicine · #Pulmonary Hypertension Research and Treatments #Cardiovascular, Neuropeptides, and Oxidative Stress Research #Liver Disease and Transplantation

paper · pdf · doi:10.1161/circresaha.113.301141

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.

Citations

• Immunosuppressive therapy in lupus‐ and mixed connective tissue disease–associated pulmonary arterial hypertension: A retrospective analysis of twenty‐three cases

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