Primary sclerosing cholangitis

In this article, evidence has been presented that immune mechanisms play a major role in the pathogenesis of PSC. Although considerable progress has been made in our understanding of the pathogenesis and treatment of this disease, many questions remain unanswered. The factors that trigger the disease in a susceptible host are unknown and it is unclear whether an exogenous agent such as retroviral infection is involved or if there is an intrinsic abnormality in the immune system. It is unknown if the pathogenetic mechanisms are the same for patients with PSC alone or patients with PSC and IBD or whether these represent separate clinical entities. Moreover it is unclear if immunosuppressants as high-dose single agents or in combination may halt or slow disease progression.