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Published on: January 16, 2013
Inflammation in pulmonary arterial hypertension
Laura C Price1, S John Wort2, Frédéric Perros3
1Faculté de Médecine, Université Paris-Sud, Kremlin Bicêtre, France; Service de Pneumologie et Réanimation Respiratoire, Centre National de Référence de l'Hypertension Artérielle Pulmonaire, Hôpital Antoine-Béclère, Assistance Publique, Hôpitaux de Paris, Clamart, France; INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie Lannelongue, Le Plessis Robinson, France; Department of Pulmonary Hypertension, National Heart and Lung Institute, Imperial College London, Royal Brompton Hospital, London, England.
Inflammation plays a key role in pulmonary arterial hypertension (PAH), a condition involving pulmonary vascular remodeling. Further research is needed to explore anti-inflammatory therapies for idiopathic PAH.
Area of Science:
- Cardiovascular Medicine
- Immunology
- Pulmonary Medicine
Background:
- Pulmonary arterial hypertension (PAH) involves pulmonary vascular remodeling and excessive cell proliferation.
- Inflammation is increasingly implicated in PAH pathophysiology, evidenced by inflammatory cell accumulation and elevated cytokines.
Purpose of the Study:
- To explore the role of inflammation in pulmonary arterial hypertension (PAH).
- To investigate the potential of anti-inflammatory therapies in treating PAH, particularly idiopathic PAH (iPAH).
Main Methods:
- Analysis of pathologic specimens from PAH patients revealing perivascular inflammatory cell infiltration.
- Measurement of circulating cytokine and chemokine levels in PAH patients.
- Review of associations between inflammatory conditions and PAH incidence.
- Assessment of the impact of treating underlying inflammatory conditions on associated PAH.
Main Results:
- PAH patient specimens show accumulation of macrophages, dendritic cells, T and B lymphocytes, and mast cells.
- Elevated circulating cytokines and chemokines correlate with worse clinical outcomes in PAH.
- Inflammatory conditions like connective tissue diseases increase PAH incidence.
- Treating underlying inflammation can alleviate associated PAH.
Conclusions:
- Inflammation is a significant factor in PAH development and progression.
- Anti-inflammatory therapies show promise, particularly for connective-tissue-disease-associated PAH.
- The efficacy of anti-inflammatory treatments in idiopathic PAH requires further investigation.
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