Medical treatment of primary biliary cirrhosis and primary sclerosing cholangitis

Digestion. 2001;64(3):137-50. doi: 10.1159/000048854.

Abstract

Treatment of primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) with ursodeoxycholic acid (UDCA) has been in common use since 1985. In PBC, treatment with UDCA improves laboratory data, liver histology, enables a longer transplantation-free interval and prolongs disease survival. Because UDCA is unable to cure the disease newer drugs or combination therapies are still needed. Studies with UDCA and immunosuppressants such as prednisone, budesonide and azathioprine have shown that in selected patients combination therapy may be superior to UDCA monotherapy. PSC is treated successfully with UDCA and endoscopic dilatation of the bile duct strictures. Treatment of extrahepatic manifestations of cholestatic liver disease such as pruritus, fatigue, osteoporosis and steatorrhea can be problematic and time-consuming.

Publication types

  • Review

MeSH terms

  • Azathioprine / therapeutic use
  • Celiac Disease / etiology
  • Celiac Disease / therapy
  • Cholagogues and Choleretics / therapeutic use*
  • Cholangitis, Sclerosing / drug therapy*
  • Cholangitis, Sclerosing / physiopathology
  • Cholangitis, Sclerosing / surgery
  • Drug Therapy, Combination
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Liver Cirrhosis, Biliary / drug therapy*
  • Liver Cirrhosis, Biliary / physiopathology
  • Osteoporosis / drug therapy
  • Osteoporosis / etiology
  • Prednisolone / therapeutic use
  • Pruritus / drug therapy
  • Pruritus / etiology
  • Tacrolimus / therapeutic use*
  • Ursodeoxycholic Acid / therapeutic use*

Substances

  • Cholagogues and Choleretics
  • Immunosuppressive Agents
  • Ursodeoxycholic Acid
  • Prednisolone
  • Azathioprine
  • Tacrolimus