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 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.
- chemokines
- connective tissue disease
- endothelial cells
- inflammatory infiltrate
- pulmonary arterial hypertension
- monocrotaline
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.
Footnotes
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↵Previous articles in this Series: No. 1: Humbert M, Trembath RC. Genetics of pulmonary hypertension: from bench to beside. Eur Respir J 2002; 20: 741–749. No. 2: Galiè N, Manes A, Branzi A. The new clinical trials on pharmacological treatment in pulmonary arterial hypertension. Eur Respir J 2002; 20: 1037–1049. No. 3: Chemla D, Castelain V, Hervé P, Lecarpentier Y, Brimioulle S. Haemodynamic evaluation of pulmonary hypertension. Eur Respir J 2002; 20: 1314–1331. No. 4: Eddahibi S, Morrell N, d'Ortho M-P, Naeije R, Adnot S. Pathobiology of pulmonary arterial hypertension. Eur Respir J 2002; 20: 1559–1572. No. 5: Widlitz A, Barst RJ. pulmonary arterial hypertension in children. Eur Respir J 2003; 21: 155–176. No. 6: Moloney ED, Evans TW. Pathophysiology and pharmacological treatment of pulmonary hypertension in acute respiratory distress syndrome. Eur Respir J 2003; 21: 720–727. No. 7: Barberà JA, Peinado, VI, Santos S. Pulmonary hypertension in chronic obstructive pulmonary disease. Eur Respir J 2003; 21: 892–905.
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