Some patients may show features of both autoimmune hepatitis and primary sclerosing cholangitis (AIH-PSC overlap syndrome). If a person with primary sclerosing cholangitis shows signs of a hepatitis, or if a person with autoimmune hepatitis appears cholestatic, then the AIH-PSC overlap syndrome should be considered.


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



• 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.


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