The specific aims of the study were: - To (re)define the clinical features of the primary biliary cirrhosis syndrome. - To develop objective criteria which combine specificity with sensitivity for the diagnosis of PBC. - To study the relation of IgM in various forms (pentameric, monomeric and cryoglobulin) to the activity of the non-suppurative destructive cholangitis. - To test the therapeutic effects of 'go-slow-golow' D-penicillamine therapy in a randomized controlled trial, and to determine whether any favourable effects were due to a reduction of hepatic copper or to a decrease of immunological disturbances. - To perform a pilot study for evaluation of the effect of a combined therapy with small doses of D-penicillamine and prednisone