Aliena Badshah, Iqbal Haider, Mohammad Humayun.
Primary Biliary Cirrhosis.
J Med Sci May ;25(1):90-5.

Primary Biliary Cirrhosis (PBC) is the result of long-standing damage to intra-hepatic biliary channels as a result of immune mechanisms leading to cholestasis and its associated complications. It can end up in chronic liver disease or cirrhosis. It usually manifests as pruritis that then proceeds to fatigue, jaundice and other features of chronic liver disease. It is detected by raised serological markers of cholestasis and presence of anti-mitochondrial antibody (AMA) in the blood. Some cases of AMA-negative PBC have also been reported though. Biopsy is diagnostic in such cases. Overlap syndromes also occur, in which case two autoimmune conditions co-exist in the same liver. These include autoimmune hepatitis (AIH) / PBC, AIH / Chronic Hepatitis C (CHC), AIH / Cryptogenic Chronic Hepatitis, and AIH / Primary Sclerosing Cholangitis (PSC) overlaps. Treatment of PBC is primarily with ursodeoxycholic acid, though steroids and steroid sparing drugs may be used in patients who show sub-clinical or no response to initial therapy with ursodeoxycholic acid.

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