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.
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. The general application of toxic immunosuppressive agents should await the results of large, long-term, prospective trials. 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.