Damage to both intra and extra hepatic bile ducts. ERCP or MRCP shows beading (segmental narrowing and dilatation)of both intra and extra hepatic ducts.
Anti smooth muscle antibodies (ASMA)(in 20%-50%), ANA, p-ANCA (not specific)
Young Man with itching, jaundice, malabsorption syndrome, signs of cirrhosis etc.
Biopsy may show periductal sclerosis (onion skinning)
Tmt: Ursodeoxycholic acid, cholestyramine, vitamin supplements
Note: ASMA and ANA + jaundice are also present in autoimmune hepatitis but Auto immune hepatitis is xterised by
extrahepatic autoimmune diseases
Treatment of autoimmune hepatitis is low dose steroids.