A case of autoimmune hepatitis/primary biliary cirrhosis overlap syndrome progressing rapidly to cirrhosis with skin pigmentation and various immunological characteristics

Abstract

A 53-year-old Japanese man was found to have a liver dysfunction with a total bilirubin of 3.3 mg/dl, ALT 724 IU/l, alkaline phosphatase 568IU/l with an elevation of serum IgM while serum levels of IgG, antinuclear antibody (ANA) and anti-mitochondrial antibody (AMA) showed normal. Primary biliary cirrhosis (PBC) was suspected at that time, however, he left the hospital by self-determination. Five years later, he developed liver cirrhosis with jaundice, ascites and skin pigmentation and sclerosis. Antinuclear antibody, negative 5 years before, was now positive at 1: more than 2560. Serum IgG and IgM were elevated. Anti-mitochondrial antibody M2 component, and liver-kidney microsome (LKM)-1 antibody, double strand DNA antibody, platelet-associated IgG, direct-, indirect Coombs tests were also positive. Liver histology revealed cirrhosis. He was diagnosed as having AIH/PBC overlap syndrome due to serologic features. Sequential changes of the titers of several antibodies, laboratory characteristics and clinical symptoms within short durations are discussed. © 2010 The Japan Society of Hepatology.