State of the art. Primary biliary cirrhosis and the immune system.

The immunologic features of diseases associated with primary biliary cirrhosis (PBC) such as Sjörgren's syndrome, scleroderma, rheumatoid arthritis and others are reviewed. Immunologic abnormalities involving both humoral and cell mediated immunity have been found in PBC. The morphologic and immunologic data have been used to propose a pathogenetic mechanism for PBC. Recent studies of penicillamine indicate that the drug may influence liver copper content and the deposition of immune complexes.