title: Immunological features of patients with primary biliary cirrhosis (PBC) overlapping systemic sclerosis: a comparison with patients with PBC alone.
abstract: To characterize the immunological features of patients with primary biliary cirrhosis (PBC) overlapping systemic sclerosis (SSc), 26 patients with PBC were classified according to the presence of scleroderma-related features (Raynaud's phenomenon, sclerodactyly etc.). The patients were classified into 10 patients with PBC overlapping SSc (PBC-SSc), four patients with some scleroderma-related features although not meeting the criteria of SSc (PBC-SSc spectrum) and 12 patients with PBC alone. Sera from PBC-SSc showed a significantly higher positivity to anti-centromere antibody (P< 0.01) and to E1beta of pyruvate dehydrogenase complex (P< 0.005) than those from patients with PBC alone. The same tendency was observed in PBC-SSc spectrum patients. Patients with PBC exhibiting scleroderma-related features, in both the PBC-SSc and PBC-SSc spectrum, may comprise a subset in PBC, not only clinically, but also immunologically.
authors.affiliation: Department of Dermatology, Gunma University School of Medicine, Japan. sakimoto@sb.gunma-u.ac.jp
authors.forename: S
authors.initials: S
authors.lastname: Akimoto
authors.forename: O
authors.initials: O
authors.lastname: Ishikawa
authors.forename: H
authors.initials: H
authors.lastname: Takagi
authors.forename: Y
authors.initials: Y
authors.lastname: Miyachi
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000032:analysis*; D002503:Centromere / Q000276:immunology*; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000145:classification / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000276:immunology*; D011768:Pyruvate Dehydrogenase Complex / Q000276:immunology*; D012595:Scleroderma, Systemic / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D011768:Pyruvate Dehydrogenase Complex