Understanding the role of CD4+CD25(high) (so-called regulatory) T cells in idiopathic pulmonary arterial hypertension.

摘要

Inflammatory mechanisms are believed to play a role in pulmonary arterial hypertension (PAH) . Indeed, severe PAH may occur in a subset of patients displaying systemic inflammatory conditions, and treatments with corticosteroids and/or immuno suppressants sometimes dramatically improve PAH complicating the POEMS syndrome (polyneuropathy, organomegaly, endocrinop-athy, and monoclonal immunoglobulin and skin changes) , Castleman's disease , systemic lupus erythema-tosus [4, 5], mixed connective tissue disease [4, 5] and primary Sjogren's syndrome . Autoimmunity and inflammation have also been demonstrated to contribute to idiopathic PAH pathogenesis [7-11]. Idiopathic PAH patients are characterized by circulating autoantibodies (antinuclear, anti-endothelial and anti-fibroblast antibodies) and elevated serum concentrations of proinflam-matory cytokines, e.g. interleukin (IL)-1 and IL-6 [7-11].