22. PRIMARY BILIARY CIRRHOSIS

Aliena Badshah, Iqbal Haider, Mohammad Humayun

Department of Medicine, Khyber Teaching Hospital, Peshawar - Pakistan

Abstract

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.

Keywords: Primary, Biliary, Cirrhosis, cholestasis, anti-mitochondrial antibody, AMA-negative primary biliary cirrhosis, overlap syndromes.

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