Liver transplantation in patients with PSC and ulcerative colitis. A clinical challenge

The survival of patients with primary sclerosing cholangitis (PSC) is reduced in part because of an increased incidence of cholangiocellular carcinoma. However, the timing of liver transplantation represents a considerable clinical challenge. The clinical course of PSC is frequently slowly progressive and can develop acutely with the occurrence of septic cholangitis. In most cases this situation is not adequately reflected by established prognostic models, including the Model for End-Stage Liver Disease (MELD) score, which is the basis for donor organ allocation in liver transplantation candidates. Therefore, an exceptional- case status has been defined for certain transplantation candidates with PSC ("Exceptional MELD"). The increased risk of cholangiocarcinoma alone does not warrant prophylactic liver transplantation. The overall results after liver transplantation for PSC are generally better than for other indications, although up to 40% develop recurrent PSC or a PSC-like syndrome. In PSC patients with cholangiocarcinoma, liver transplantation should not be performed outside of clinical studies. The prevalence of chronic inflammatory bowel disease after liver transplantation in patients with PSC is reported to exceed 65% (up to 100%) and is usually classified as ulcerative colitis. Proctocolectomy can be safely performed after transplantation. However, the extent to which patients have an improved course of recurrent PSC after proctocolectomy has yet to be established.