TY - JOUR T1 - Inflammation in pulmonary arterial hypertension JF - European Respiratory Journal JO - Eur Respir J SP - 358 LP - 363 DO - 10.1183/09031936.03.00038903 VL - 22 IS - 2 AU - P. Dorfmüller AU - F. Perros AU - K. Balabanian AU - M. Humbert Y1 - 2003/08/01 UR - http://erj.ersjournals.com/content/22/2/358.abstract N2 - Inflammatory mechanisms appear to play a significant role in some types of pulmonary hypertension (PH), including monocrotaline-induced PH in rats and pulmonary arterial hypertension of various origins in humans, such as connective tissue diseases (scleroderma, systemic lupus erythematosus, mixed connective disease), human immunodeficiency virus infection, or plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, monoclonal (M) protein and skin changes (POEMS) syndrome. Interestingly, some patients with severe pulmonary arterial hypertension associated with systemic lupus erythematosus have experienced significant improvements with immunosuppressive therapy, emphasising the relevance of inflammation in a subset of patients presenting with PH. Patients with primary PH (PPH) also have some immunological disturbances, suggesting a possible role for inflammation in the pathophysiology of this disease. A subset of PPH patients have been shown to have circulating autoantibodies, including antinuclear antibodies, as well as elevated circulating levels of the pro-infammatory cytokines, interleukins ‐1 and ‐6. Lung histology has also revealed inflammatory infiltrates in the range of plexiform lesions in patients displaying severe PPH, as well as an increased expression of the chemokines regulated upon activation, normal T‐cell expressed and secreted (RANTES) and fractalkine. Further analysis of the role of inflammatory mechanisms is necessary to understand whether this component of the disease is relevant to its pathophysiology. This group has been supported in part by grants from Legs Poix, Université Paris-Sud, Association Française contre les Myopathies and Institut National de la Santé et de la Recherche Médicale. ER -