Medical treatment of primary biliary cirrhosis and primary sclerosing cholangitis.
Chronic cholestasis is the main feature of primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), the most common chronic cholestatic liver diseases in adults. Although the etiology of both diseases remains poorly understood, auto-immune processes appear to be important, particularly in PBC. PBC and PSC usually slowly progress to cirrhosis,liver failure, and death, unless liver transplantation is performed. Ursodeoxycholic acid(UDCA), a hydrophilic dihydroxy bile acid, is the only drug currently approved for the treatment of patients with PBC and is also used in patients with PSC. In addition to UDCA, patients with PSC should be referred to endoscopic dilatation of major bile duct stenoses. Several potential mechanisms of action of UDCA have been proposed, including intracellular modulation of signaling events and secretion. Various immunosuppressive drugs have been evaluated alone or in combination with UDCA-especially for the treatment of PBC. Of these drugs,the topical corticosteroid budesonide, together with UDCA, appears promising in the treatment of early stage PBC, but data remain insufficient to warrant use of budesonide outside of controlled studies.
Rust
Christian
C
Department of Internal Medicine II Grosshadern, University of Munich, Munich, Germany. Christian.Rust@med.uni-muenchen.de
Beuers
Ulrich
U
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D017209:Apoptosis; D015209:Cholangitis, Sclerosing / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy*; D011239:Prednisolone / Q000627:therapeutic use; D014580:Ursodeoxycholic Acid / Q000493:pharmacokinetics / Q000627:therapeutic use
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid; D011239:Prednisolone