TY - T1的抗体在idiopa成纤维细胞thic and scleroderma-associated pulmonary hypertension JF - European Respiratory Journal JO - Eur Respir J SP - 799 LP - 807 DO - 10.1183/09031936.06.00152705 VL - 28 IS - 4 AU - Tamby, M. C. AU - Humbert, M. AU - Guilpain, P. AU - Servettaz, A. AU - Dupin, N. AU - Christner, J. J. AU - Simonneau, G. AU - Fermanian, J. AU - Weill, B. AU - Guillevin, L. AU - Mouthon, L. Y1 - 2006/10/01 UR - //www.qdcxjkg.com/content/28/4/799.abstract N2 - The aim of the present study was to investigate the presence of anti-fibroblast antibodies in patients with idiopathic or scleroderma-associated pulmonary arterial hypertension (PAH) and healthy controls. PAH was documented by right-heart catheterisation (mean pulmonary artery pressure at rest >25 mmHg). Serum immunoglobulin (Ig)G and IgM reactivities of patients with idiopathic PAH (n = 35), scleroderma-associated PAH (n = 10), diffuse (n = 10) or limited cutaneous (n = 10) scleroderma without PAH and age- and sex-matched healthy individuals (n = 65) were analysed by cell-based ELISA and immunoblotting on normal human fibroblasts. As assessed by ELISA, 14 out of 35 (40%) patients with idiopathic PAH and three out of 10 (30%) patients with scleroderma-associated PAH expressed anti-fibroblast IgG antibodies. IgG from all individuals bound to one major 40-kDa protein band. IgG from patients with idiopathic PAH bound to two 25- and 60-kDa bands with a higher intensity than IgG from other individuals. In conclusion, immunoglobulin G anti-fibroblast antibodies are present in the serum of patients with pulmonary arterial hypertension. Immunoglobulin G from patients with idiopathic pulmonary arterial hypertension or scleroderma-associated pulmonary arterial hypertension express distinct reactivity profiles with fibroblasts antigens, suggesting distinct target antigens. ER -