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PRIMARY BILIARY CHOLANGITIS

Primary biliary cholangitis (formerly primary biliary cirrhosis) is a chronic, progressive, autoimmune, cholestatic liver disease more common in middle-aged women. It is characterized by destruction of small to medium bile ducts leading to cholestasis and frequently, end-stage liver disease.
Diagnostic features are chronic biochemical cholestasis, presence of antimitochondrial antibodies and the characteristic liver biopsy findings.


 

Surgical Intervention

Liver Transplantation 

  • Liver transplantation is the only life-saving procedure for patients with progressive PBC and consequent liver failure
    • Increasing bilirubin and decreasing albumin levels and a prolonged prothrombin time are signs of disease progression
  • A referral for a liver transplant evaluation should be done in patients with decompensated cirrhosis, total bilirubin >6 mg/dL, a MELD score of at least 15 and >7.8 from the updated Mayo Clinic Natural History Model for PBC 
  • Consider liver transplantation in refractory ascites, recurrent spontaneous bacterial peritonitis, recurrent variceal bleeding, hepatic coma, HCC or hepatorenal syndrome type I
    • Uncontrollable pruritus resistant to medical therapy may be an indication for liver transplantation
  • Patients with PBC recurrence post liver transplant can be given UDCA therapy
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