Primary Biliary Cirrhosis (PBC) is a chronic and progressive cholestatic disease of the liver. The etiology is unknown, although it is presumed to be autoimmune in nature. The major pathology of this disease is a destruction of the small-to-medium bile ducts, which leads to progressive cholestasis and often end-stage liver disease.
Important notes:
Ursodeoxycholic acid (UDCA) is the major medication used to slow the progression of the disease. Patients with early disease have clinical, biochemical, and histologic improvement. Reports suggest that UDCA delays the need for transplantation or delays death. The efficacy of this medication in late stages (ie, cirrhosis) is questionable. Patients who achieve biochemical response to UDCA after 1 year of treatment reportedly have a similar survival rate to the matched control population, and this observation might be used to identify the population of nonresponders who will require alternative or additional treatments. The use of UDCA after transplantation with evidence of recurrence of the disease has been associated with biochemical response, although its role in delaying histologic progression needs further investigation.
As the disease progresses to cirrhosis, an elevated bilirubin level, a prolonged prothrombin time, and a decreased albumin level can be found. The increased bilirubin level is an ominous sign of disease progression, and liver transplantation must be considered. Liver transplantation appears to be the only life-saving procedure.
