Anti-DNA antibody idiotypes in primary biliary cirrhosis

• Published in: Cellular immunology Vol. 104; no. 1; pp. 37 - 46
• Main Authors: Schattner, A., Kaburaki, Y., Kaplan, M.M., Miller, K.B.
• Format: Journal Article
• Language: English
• Published: San Diego, CA Elsevier Inc 1987; Elsevier
• Subjects: Adult; Autoantibodies - analysis; Autoimmunity (experimental aspects and models); Biological and medical sciences; DNA - immunology; DNA, Single-Stranded - immunology; Female; Fundamental and applied biological sciences. Psychology; Fundamental immunology; Humans; Immunoglobulin Idiotypes - analysis; Liver Cirrhosis, Biliary - genetics; Liver Cirrhosis, Biliary - immunology; Lupus Erythematosus, Systemic - immunology; Male; Middle Aged; Autoimmunity; Human; Antibody; DNA; Autoantibody; Lupus erythematosus; Autoimmune disease; Biliary cirrhosis; Idiotype
• ISSN: 0008-8749; 1090-2163
• DOI: 10.1016/0008-8749(87)90004-9

A significant increase in 16/6 Id—a major cross-reactive idiotype of anti-DNA antibodies (Ab) derived from a patient with systemic lupus erythematosus (SLE) and hitherto identified in SLE patients and their relatives, was found in 16/17 patients with primary biliary cirrhosis (PBC). The increased serum level of Ab with the 16/6 idiotype (16/6 Id) in PBC patients (median 50 ng/ml) was not found in 6/7 of the patients' spouses nor among 27/28 healthy controls or most patients with other types of cirrhosis. The quantity of 16/6 Id was not correlated to either the stage of disease or the presence of antimitochondrial, antinuclear, or anti-dsDNA antibodies. However, 16/6 Id could be shown to be associated with anti-ssDNA antibodies. The high frequency of the lupus-derived 16/6 Id in PBC may accompany the polyclonal B-cell activation seen in that disease. Of 14 healthy first-degree relatives of the PBC patients, 4 (29%) also had elevated serm 16/6 Id (20–25 ng/ml) and the cluster of 3 of them in a single family may indicate a genetic predisposition to develop PBC.