Immunosuppressive treatment of primary sclerosing cholangitis current status and recommendations

Primary sclerosing cholangitis (PSC) is a disease of unknown aetiology characterised by focal as well as diffuse destruction of bile ducts. The disease frequently, but not always, occurs in association with an inflammatory bowel disease, usually ulcerative colitis and less frequently Crohn's disease. A small number of cases occur in the absence of a concomitant colonie disease. In addition to being associated with inflammatory bowel disease. PSC occurs predominantly in individuals who are HLA-B8, Dr3. DW52.53 positive. These same HLA antigens occur at increased rates in individuals with other putative autoimmune diseases. As a result of these disease and genetic associations, PSC is often conceptualised as an autoimmune disease process. For this reason, immunosuppressive agents have been utilised and currently are the principal agents used in the treatment of patients with PSC. The rationale forthe use of these agents, their efficacy, administration schedules and the risks associated with their use are presented. In general, individuals with early stage disease should be treated with immunosuppressive agents that disrupt the pathogenesis of the disease process. In contrast, late stage disease that is effectively irreversible ought to be treated symptomatically with agents that alleviate disease consequence. Adis International Limited Ail nghts reserved.