Thursday, May 10, 2012

Primary sclerosing cholangitis

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
female preponderance,
extrahepatic autoimmune diseases
hypergammaglobulinemia.

Treatment of autoimmune hepatitis is low dose steroids.

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