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Clinical Reviews in Allergy & Immunology
Published in: Clinical Reviews in Allergy & Immunology 1/2013

01-02-2013

The Role of Inflammation and Autoimmunity in the Pathophysiology of Pulmonary Arterial Hypertension

Authors: Nada Kherbeck, Mathieu C. Tamby, Guillaume Bussone, Hanadi Dib, Frederic Perros, Marc Humbert, Luc Mouthon

Published in: Clinical Reviews in Allergy & Immunology | Issue 1/2013

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Abstract

Pulmonary arterial hypertension is characterized by a remodeling of pulmonary arteries with endothelial cell, fibroblast, and vascular smooth muscle cell activation and proliferation. Since pulmonary arterial hypertension occurs frequently in autoimmune conditions such as systemic sclerosis, inflammation and autoimmunity have been suspected to play a critical role in both idiopathic pulmonary arterial hypertension and systemic sclerosis-associated pulmonary arterial hypertension. High levels of pro-inflammatory cytokines such as interleukin-1 and interleukin-6, platelet-derived growth factor, or macrophage inflammatory protein 1 have been found in lung samples of patients with pulmonary arterial hypertension, along with inflammatory cell infiltrates mainly composed of macrophages and dendritic cells, T and B lymphocytes. In addition, circulating autoantibodies are found in the peripheral blood of patients. Thus, autoimmunity and inflammation probably play a role in the development of pulmonary arterial hypertension. In this setting, it would be important to set-up new experimental models of pulmonary arterial hypertension, in order to define novel therapeutics that specifically target immune disturbances in this devastating condition.
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Metadata
Title
The Role of Inflammation and Autoimmunity in the Pathophysiology of Pulmonary Arterial Hypertension
Authors
Nada Kherbeck
Mathieu C. Tamby
Guillaume Bussone
Hanadi Dib
Frederic Perros
Marc Humbert
Luc Mouthon
Publication date
01-02-2013
Publisher
Humana Press Inc
Published in
Clinical Reviews in Allergy & Immunology / Issue 1/2013
Print ISSN: 1080-0549
Electronic ISSN: 1559-0267
DOI
https://doi.org/10.1007/s12016-011-8265-z

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