Korean J Pathol.  1997 Jan;31(1):87-90.

Primary Biliary Cirrhosis-Autoimmune Hepatitis Overlap Syndrome

• Bae JY
• Park YN
• Park C

Abstract

Cases sharing features of both primary biliary cirrhosis and autoimmune hepatitis have been reported as a mixed type, overlap syndrome, immunocholangitis and autoimmune cholangiopathy. A primary biliary cirrhosis- autoimmune hepatitis overlap syndrome is unusual and characterized by overlapping features; cholestasis, high titer of alkaline phosphatase, bile duct damage and granulomas in the liver biopsy, high antinuclear antibody, increased IgG and IgM and intra-acinar hepatitis with piecemeal necrosis. Autoimmune mechanisms are thought to play a major role in the pathogenesis of the overlapping syndrome and the bases of immunosuppressive therapy. A 58-year-old female patient shows overlapping clinical and laboratory findings, chronic active hepatitis in initial liver biopsy which transits to primary biliary cirrhosis with cholangitis and granulomas. This is a case of hepatobiliary lesion showing overlapping features of both primary biliary cirrhosis and autoimmune hepatitis over 3-year period.

Keyword

Overlap syndrome; Primary biliary cirrhosis; Autoimmune hepatitis

MeSH Terms

Alkaline Phosphatase
Antibodies, Antinuclear
Bile Ducts
Biopsy
Cholangitis
Cholestasis
Female
Granuloma
Hepatitis*
Hepatitis, Autoimmune
Hepatitis, Chronic
Humans
Immunoglobulin G
Immunoglobulin M
Liver
Liver Cirrhosis, Biliary
Middle Aged
Necrosis
Alkaline Phosphatase
Antibodies, Antinuclear
Immunoglobulin G
Immunoglobulin M