Cited by 1,029Pulmonary Hypertension Research and TreatmentsCardiac Fibrosis and RemodelingSystemic Sclerosis and Related Diseases

Inflammation and immunity in the pathogenesis of pulmonary arterial hypertension

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Rabinovitch, Marlene, Guignabert, Christophe, Humbert, Marc, Nicolls, Mark R.·2014·Circulation Research

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.

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Crossref · 10.1161/circresaha.113.301141

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  1. [126]Cracowski JL Chabot F Labarere J Faure P Degano B Schwebel C Chaouat A Reynaud-Gaubert M Cracowski C Sitbon O Yaici A Simonneau G Humbert M Proinflammatory cytokine levels are linked with death in pulmonary arterial hypertension Eur Respir J 2013 24232704

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Authors
Rabinovitch, M., Guignabert, C., Humbert, M., & Nicolls, M. R.
Affiliations
Université Paris-Sud (FR)
Venue
Circulation Research
Published
2014-06-20
Volume
115
Issue
1
Pages
165-175
ISSN
  • 0009-7330 (ISSN-L)
  • 1524-4571
OpenAlex
W2132001965
Cited by
1,029
References
211
Citations by year
2026: 78 · 2025: 84 · 2024: 114 · 2023: 84 · 2022: 102 · 2021: 141 · 2020: 92 · 2019: 101 · 2018: 68 · 2017: 60 · 2016: 62 · 2015: 34 · 2014: 6 · 2013: 1 · 2012: 1
FWCI
43.24
Type
journal article
Language
en
Open access
Locations
  • Circulation Research (publishedVersion)
  • PubMed (publishedVersion)
  • Europe PMC (PubMed Central) (acceptedVersion)
  • PubMed Central (acceptedVersion)
Funders
  • Agence Nationale de la Recherche (ANR-12-JSV1-0004)
  • National Institutes of Health (5r01hl074186-17, 5p01hl108797-10)
  • National Heart, Lung, and Blood Institute (HL082662, R01-HL074186, R01HL095686, R56HL082662, R01HL082662, P01-HL-108797)
Topics
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Fibrosis and Remodeling
  • Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
Keywords
pulmonary arterial hypertension, inflammation, immune dysregulation, chemokines, cytokines, biomarkers, animal models, anti-inflammatory therapy, pulmonary vascular remodeling, genetic abnormalities, metabolic abnormalities
MeSH terms
Animals, Dendritic Cells, T-Lymphocytes, Helper-Inducer, Humans, Hypertension, Pulmonary, Immunity, Inflammation, Lymphocytes, Macrophages, Biomarkers, Cytokines, Chemokines, Familial Primary Pulmonary Hypertension

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