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Wednesday, January 23, 2008

Primary Biliary Cirrhosis

Primary Biliary Cirrhosis (PBC)

World wide incidence is 4-5 / million (middle age women).
Suspected genetic component in causation (6 fold increase in frequency of DRW8 tissue type among PBC patients).
Other autoimmune diseases such as arthritis, thyroiditis, keratoconjunctivitis, kidney and skin disease occurs with PBC.
Patients present with fatigue and itching and progresses over 10-15 years, presenting with progressive jaundice.

Serum alkaline phosphatase is raised.
Serum IgM titer and Cholesterol are raised.
95% have raised Anti mitochondrial antibody titer.
Liver biopsy and histo pathology study will show chronic inflammation of bile ductules around portal tract, granulomatous destruction of bile ducts, liver cell death in early stages and later fibrosis and disappearing bile ducts lead to frank cirrhosis.

Drugs such as Azathioprine, Steroids, Chlorambucil and Cyclosporin can produce biochemical improvement at the cost of bad side effects.
Drugs such as UDCA (Ursodeoxycholic acid) and methotrexate are more promising as they produce biochemical and clinical improvement of symptoms.
No known medical therapy can increase patient survival.
Liver transplantation has the best chance at cure (80% survival at 1 year), all PBC patients with bilurubin > 5 mg/d1 should enroll for liver transplantation.

Progression of disease stretches over 10 to 15 years.
Older age group, enlarged liver and level of bilurubin correlates with shorter life span
Serum bilurubin level > 2mg/d1 indicates a mean survival of 4 years.
Small percentage can develop recurrent PBC after liver transplantation.

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