TY - JOUR
T1 - Medical treatment of primary biliary cirrhosis and primary sclerosing cholangitis
AU - Rust, Christian
AU - Beuers, Ulrich
PY - 2005
Y1 - 2005
N2 - 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
AB - 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
U2 - https://doi.org/10.1385/CRIAI:28:2:135
DO - https://doi.org/10.1385/CRIAI:28:2:135
M3 - Review article
C2 - 15879619
SN - 1080-0549
VL - 28
SP - 135
EP - 145
JO - Clinical reviews in allergy & immunology
JF - Clinical reviews in allergy & immunology
IS - 2
ER -