Primary biliary cirrhosis associated with benign monoclonal gammopathy

Abstract

A 75-year-old man was diagnosed as asymptomatic primary biliary cirrhosis (PBC) on the basis of detected anti-pyruvate dehydrogenase E2 antibody and histopathological findings compatible with chronic non-suppurative destructive cholangitis. This was associated with benign monoclonal gammopathy (BMG) of IgM- κ type. Only a few cases of PBC in association with IgM-type BMG have been reported. We present this case of rare association and discuss the possible mechanisms of M-proteinemia in aged patients with PBC.