Features suggesting primary sclerosing cholangitis:
(1) presence of inflammatory bowel disease
(2) biochemical evidence of cholestasis with elevated serum alkaline phosphatase (serum alkaline phosphatase may be normal in pediatric patients)
(3) cholangiographic changes of sclerosing cholangitis (fibrotic strictures of large bile ducts, may be subtle in pediatric patients)
(4) liver biopsy showing bile duct injury (vanishing bile ducts or concentric periductal fibrosis)
(5) often pANCA positive
Features suggesting autoimmune hepatitis:
(1) probable or definite score on the revised IAHG score (above)
(2) serum ALT > 5 times normal
(3) elevated IgG and/or gamma globulins
(4) ANA or anti-SMA at a titer >= 1:40
(5) liver biopsy with moderate to severe interface hepatitis (piecemeal necrosis), lymphocytic rosetting and moderate to severe periportal or periseptal lymphoplasmacytic inflammation
where:
• Most of the features under autoimmune hepatitis are scored in the revised IAHG score.
The AIH-PSC overlap syndrome should be considered when a patient has several features from each of the 2 conditions.
