Primary Biliary Cirrhosis in MRCP

Primary Biliary Cirrhosis (PBC)

PBC is an autoimmune disease. It is characterized by progressive inflammmation and destruction of of the small bile ducts within the liver. In the long run, it can lead to liver cirrhosis and PBC is one of the important causes of liver cirrhosis among female patients. ( 90% of PBC patients are female!)

Clinical Presentations
Usually asymptomatic but later due to biliary obstruction, patients tend to have pruritus and jaundice. Patients may present late with symptoms of liver cirrhosis such as upper GI bleeding or abdominal distention ( ascites)

Physical signs
Hepatosplenomegaly, Jaundice, scratch marks, xanthelasma, clubbing or other signs suggesting associated autoimmune diseases. Click here to see xanthelasma!

Associations
Autoimmune diseases such as Sicca syndrome, thyroiditis, RA, SLE

Investigations
I think the single test that can give a clue of PBC is LFT- you would notice markedly high ALP with raised bilirubin, IgM may be raised.
ANA may be positive
Diagnosis can be made by detecting antimitochondrial antibody. Specificity of the M2 subtype is 95-99%
Liver biopsy shows granulomas

Treatment
symtomatic relief of pruritus by cholestyramine.
Ursodeoxycholic acid may be useful
Cochicine may be used to slow down the progression
Immunosuppresants such as steroid, methotrexate may be useful
The only cure- liver transplantation

Tips for MRCP
1) Remember that if a lady has jaundice and very high ALP with pruritus, suspect PBC