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Inflammation in Pulmonary Arterial HypertensionInflammation in Pulmonary Arterial Hypertension

Laura C. Price, MBChB; S. John Wort, MBChB, PhD; Frédéric Perros, PhD; Peter Dorfmüller, MD, PhD; Alice Huertas, MD, PhD; David Montani, MD, PhD; Sylvia Cohen-Kaminsky, PhD; Marc Humbert, MD, PhD
Author and Funding Information

From Faculté de Médecine (Drs Price, Perros, Dorfmüller, Huertas, Montani, Cohen-Kaminsky, and Humbert), Université Paris-Sud, Kremlin Bicêtre, France; Service de Pneumologie et Réanimation Respiratoire (Drs Price, Perros, Dorfmüller, Huertas, Montani, Cohen-Kaminsky, and Humbert), Centre National de Référence de l’Hypertension Artérielle Pulmonaire, Hôpital Antoine-Béclère, Assistance Publique, Hôpitaux de Paris, Clamart, France; INSERM U999 (Drs Price, Perros, Dorfmüller, Huertas, Montani, Cohen-Kaminsky, and Humbert), Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie Lannelongue, Le Plessis Robinson, France; and the Department of Pulmonary Hypertension (Drs Price and Wort), National Heart and Lung Institute, Imperial College London, Royal Brompton Hospital, London, England.

Correspondence to: Marc Humbert, MD, PhD, Service de Pneumologie et Réanimation Respiratoire, Centre National de Référence de l’Hypertension Pulmonaire Sévère, Hôpital Antoine Béclère, Assistance Publique Hôpitaux de Paris, Université Paris-Sud 11, 157, Rue de la Porte de Trivaux, 92140 Clamart, France; e-mail: marc.humbert@abc.aphp.fr


Drs Price and Wort contributed equally to this article.

Funding/Support: Dr Price receives funding from the British Heart Foundation. Dr Perros is supported by the FRM [Grant DEQ20100318257]. Drs Montani and Dorfmüller are supported by a grant from Association HTAPFrance.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2012 American College of Chest Physicians


Chest. 2012;141(1):210-221. doi:10.1378/chest.11-0793
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Pulmonary arterial hypertension (PAH) is characterized by pulmonary vascular remodeling of the precapillary pulmonary arteries, with excessive proliferation of vascular cells. Although the exact pathophysiology remains unknown, there is increasing evidence to suggest an important role for inflammation. Firstly, pathologic specimens from patients with PAH reveal an accumulation of perivascular inflammatory cells, including macrophages, dendritic cells, T and B lymphocytes, and mast cells. Secondly, circulating levels of certain cytokines and chemokines are elevated, and these may correlate with a worse clinical outcome. Thirdly, certain inflammatory conditions such as connective tissue diseases are associated with an increased incidence of PAH. Finally, treatment of the underlying inflammatory condition may alleviate the associated PAH. Underlying pathologic mechanisms are likely to be “multihit” and complex. For instance, the inflammatory response may be regulated by bone morphogenetic protein receptor type 2 (BMPR II) status, and, in turn, BMPR II expression can be altered by certain cytokines. Although antiinflammatory therapies have been effective in certain connective-tissue-disease-associated PAH, this approach is untested in idiopathic PAH (iPAH). The potential benefit of antiinflammatory therapies in iPAH is of importance and requires further study.

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