YOU ARE NOW CONNECTED TO THE TOXLINE (1981 FORWARD, NON-ROYALTY) FILE. ==PSC - PRIMARY SCLEROSING CHOLANGITIS== 3 AUTHOR Amor A AUTHOR Chapoutot C AUTHOR Michel J AUTHOR Pageaux GP AUTHOR Larrey D AUTHOR Michel H TITLE [Secondary sclerosing cholangitis] SOURCE Presse Med; VOL 24, ISS 20, 1995, P948-52 (REF: 23) ABSTRACT Secondary sclerosing cholangitis leads to slow and often irreversible destruction of the walls of both intra- and extrahepatic bile ducts. As for primary sclerosing cholangitis, clinical signs and laboratory findings reveal cholestasis. The diagnosis is confirmed by retrograde endoscopic cholangiography which shows narrowed bile ducts and rarefied ramifications of the intra-hepatic ductal system. Several causes have been identified including infectious causes with or without a relationship to bile duct obstruction and human immunodeficiency virus infection as well as ischaemic related causes after chemotherapy, arterial embolization or liver transplantation. Other causes include chemical aggression after treatment for hydatic cysts and post-surgical complications due to a damaged bile tract. Treatment is difficult and often dependent on the cause. 11 AUTHOR Fennerty MB TITLE Primary sclerosing cholangitis and primary biliary cirrhosis. How effective is medical therapy? SOURCE Postgrad Med; VOL 94, ISS 6, 1993, P81-8; 92 (REF: 38) ABSTRACT Although a great deal of investigation has led to some novel approaches in treating primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC), no method has been proven to definitively alter the long-term outcome of either disease. In addition, many of the medical regimens reviewed in this article have serious toxicity, which limits their widespread use. Furthermore, the natural history of PSC and PBC may vary widely between individual patients, complicating the decision of whom and when to treat. Ursodeoxycholic acid (Actigall) appears to be a safe and possibly effective agent for both diseases in their early stages, and its use in selected patients may be warranted. Methotrexate (Folex, Mexate, Rheumatrex Dose Pack) may improve liver enzyme levels in some patients, but it is hepatotoxic and should be used cautiously. The general application of toxic immunosuppressive agents should await the results of large, long-term, prospective trials. Liver transplantation is the only proven effective therapy for advanced, decompensated PSC and PBC. 13 AUTHOR Stiehl A TITLE [Treatment of cholestatic liver diseases; the role of ursodeoxycholic acid] SOURCE Z Gastroenterol; VOL 30, ISS 10, 1992, P743-7 (REF: 45) ABSTRACT Ursodeoxycholic acid (UDCA) allows symptomatic treatment of cholestatic liver diseases such as primary biliary cirrhosis, primary sclerosing cholangitis, intrahepatic biliary atresia, and cholestasis of cystic fibrosis. Patients should be treated at an early stage of the disease in order to prevent progression to cirrhosis. Since UDCA has no toxic effects longterm treatment with this substance is possible without the risk of undesired side effects. In patients with primary biliary cirrhosis and rapid progression of the disease, UDCA may be combined with an immunosuppressive substance (i.e. cyclosporin). In primary sclerosing cholangitis, biliary atresia and cholestasis of cystic fibrosis, UDCA at present seems the only treatment of which a benefit for the patients can be expected. In endstage disease liver transplantation is indicated. The role of UDCA in chronic hepatitis and alcohol induced liver disease needs to be clarified in further studies. Whether the improvement of laboratory tests in such patients indicates amelioration of the course of disease, still is unclear. 17 AUTHOR Lindor KD AUTHOR Wiesner RH AUTHOR MacCarty RL AUTHOR Ludwig J AUTHOR LaRusso NF TITLE Advances in primary sclerosing cholangitis. SOURCE Am J Med; VOL 89, ISS 1, 1990, P73-80 (REF: 83) ABSTRACT Primary sclerosing cholangitis is an increasingly recognized chronic cholestatic liver disease. It frequently occurs in association with chronic ulcerative colitis and is characterized by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. The cause is unknown, although many mechanisms have been considered, including infectious, toxic, and immunologic. The prognosis varies. No adequate treatment exists, although a number of potential treatments have been evaluated in uncontrolled trials, and the results of controlled trials have only recently been reported. Liver transplantation has recently been shown to be an effective treatment for end-stage disease. These various advances in our understanding of primary sclerosing cholangitis are reviewed.