Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy

Table 1 Primary biliary cirrhosis - autoimmune hepatitis overlap can present in the following ways

Immunoserological overlap: e.g., positive ANA/anti-smooth muscle antibody titers and elevated IgG in conjunction with AMA-positive PBC; or AMA positivity in AIH

Biochemical overlap: AST/ALT > 5 times upper limit of normal in patients with PBC or PSC; or AP > 3 times upper limit of normal in patients with AIH (or GGT > 5 times upper limit of normal in children)

Radiological overlap: clinical features of AIH with cholangiographic abnormalities indicative of inflammatory cholangiopathy; cholangiographic features of primary sclerosing cholangitis are randomly distributed annular strictures out of proportion to upstream dilatation[33]

Histological overlap: lymphoplasmacytic infiltrate and interface hepatitis on liver biopsy with bile duct lesions indicative of either PBC or PSC

Varying combinations of the above, including sequential presentations

ANA: Antinuclear antibodies; PBC: Primary biliary cirrhosis; AMA: Anti-mitochondrial antibodies; AIH: Autoimmune hepatitis; PSC: Primary sclerosing cholangitis; AST: Aspartate aminotransferase; ALT: Alanine aminotransferase; GGT: Gamma glutamyl transpeptidase.