Inflammation and immunity in the pathogenesis of pulmonary arterial hypertension

Circ Res. 2014 Jun 20;115(1):165-75. 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.

Keywords: hypertension, pulmonary; leukotriene B4.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Biomarkers
  • Chemokines / physiology
  • Cytokines / physiology
  • Dendritic Cells / immunology
  • Familial Primary Pulmonary Hypertension
  • Humans
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / immunology*
  • Hypertension, Pulmonary / pathology
  • Hypertension, Pulmonary / therapy
  • Immunity*
  • Inflammation / complications*
  • Lymphocytes / immunology
  • Macrophages / physiology
  • T-Lymphocytes, Helper-Inducer / immunology

Substances

  • Biomarkers
  • Chemokines
  • Cytokines