Presse Med. 2011 Apr;40 Suppl 1:1S3-13.

Novel immunopathological approaches to pulmonary arterial hypertension.

Perros F, Montani D, Dorfmüller P, Huertas A, Chaumais MC, Cohen-Kaminsky S, Humbert M.

[Article in French]

Université Paris-Sud, Faculté de médecine, Le Kremlin-Bicêtre, France.

Abstract

Inflammation is important for the initiation and the maintenance of vascular remodeling in the most commun animal models of pulmonary hypertension (PH), and its therapeutical targeting blocks PH development in these models. In human, pulmonary vascular lesions of PH are also the source of an intense chemokine production, linked to inflammatory cell recruitment. However, arteritis is uncommon in PH patients. Of note, current PH treatments have immunomodulatory properties. In addition, some studies have shown a correlation between levels of circulating inflammatory mediators and patients' survival. The study of autoimmunity in the pathophysiology of pulmonary arterial hypertension is becoming an area of intense investigation. New immunopathological approaches to PH should allow the development of innovative treatments for this very severe condition.

PMID:21536178