| [ Summary ] |
Ursodeoxcholic acid delays histologic progression of patients with primay biliary cirrhosis (PBC) when given in the early stages of the condition. However, the most effective therapeutic alternative for patients with end-stage PBC is liver transplantation. Indications for referral include variceal bleeding, ascites requiring frequent large volume paracentesis, hepatic encephalopathy, spontaneous bacterial peritonitis, hepatorenal syndrome, hepatopulmonary syndrome, severe fatigue, intractable pruritus, and severe pain from vertebral body compression fractures. In order to define the optimal time for liver transplantation, the Child-Pugh score, the MELD score, the Mayo risk score or death probability ditermined by guidelines of the Japanese Liver Transplantation Society are useful. Graft and patient survival rates are better compared to rates for patients receiving transplants because of HCV or alcoholic liver cirrhosis. One-year, five-year and ten-year survival rates for PBC are estimated to be 80 %, 75 %, and 70 %, respectively. PBC recurs in about 9-35 % of patients. A diagnosis of recurrent PBC can be reliably established only through histologic examination. To clarify the actual frequency and outcomes associated with recurrent PBC, continued follow-up is essential. |