title: Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension.
abstract: 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.
authors.affiliation: Paris-Descartes University, Faculty of Medicine, Paris, France.
authors.forename: M C
authors.initials: MC
authors.lastname: Tamby
authors.forename: M
authors.initials: M
authors.lastname: Humbert
authors.forename: P
authors.initials: P
authors.lastname: Guilpain
authors.forename: A
authors.initials: A
authors.lastname: Servettaz
authors.forename: N
authors.initials: N
authors.lastname: Dupin
authors.forename: J J
authors.initials: JJ
authors.lastname: Christner
authors.forename: G
authors.initials: G
authors.lastname: Simonneau
authors.forename: J
authors.initials: J
authors.lastname: Fermanian
authors.forename: B
authors.initials: B
authors.lastname: Weill
authors.forename: L
authors.initials: L
authors.lastname: Guillevin
authors.forename: L
authors.initials: L
authors.lastname: Mouthon
mesh_terms: D000328:Adult; D002460:Cell Line; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005347:Fibroblasts / Q000276:immunology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000276:immunology*; D015151:Immunoblotting; D007074:Immunoglobulin G / Q000097:blood*; D007075:Immunoglobulin M / Q000097:blood*; D008297:Male; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000150:complications / Q000276:immunology*
chemical_list: D007074:Immunoglobulin G; D007075:Immunoglobulin M