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Inflammation in pulmonary arterial hypertension

Inflammation in pulmonary arterial hypertension,10.1183/09031936.03.00038903,European Respiratory Journal,P. Dorfmuller,F. Perros,K. Balabanian,M. Hum

Inflammation in pulmonary arterial hypertension   (Citations: 158)
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ABSTRACT: 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 immuno- logical 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. Eur Respir J 2003; 22: 358‐363. Centre,for,Pulmonary,Vascular,Diseases UPRES EA 2705, Dept of Respiratory and Intensive Care Medicine, South Paris Cytokine Institute, Antoine Be ´cle `re Hospital, Assistance Publique-Ho ˆ pitaux de Paris, South Paris University, Clamart, France. Correspondence: M. Humbert, Dept of
Journal: European Respiratory Journal - EUR RESP J , vol. 22, no. 2, pp. 358-363, 2003
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