Survival rates are comparable with those achieved in patients with other autoimmune liver diseases, such as primary biliary cirrhosis and autoimmune hepatitis, with 1-year patient survival exceeding 90 % in recently published series . PSC patients are reported to have a slightly higher retransplantation rate without impact on long-term patient survival . Again, this increased retransplantation rate has been ascribed to PSC-specific long-term complications, in particular, biliary strictures . However, patient survival following Ltx has been shown to be clearly improved compared with the natural course of the disease predicted by prognostic models .
A number of pretransplantation factors have been shown to predict poorer posttransplantation survival, such previous hepatobiliary surgery, elevated creatinine levels, reduced nutritional status and the occurrence of hepatobiliary malignancy [4,18]. Presence of IBD has also been claimed to affect survival following Ltx . Other studies failed to identify IBD as a risk factor . The greatest impact on long-term outcome is from CCC, which has been considered poor even in patients with occult tumours [19, 20].
Considering the lack of alternatives and the significant benefit in patients with small tumours, strict exclusion from transplantation is not justified. Moreover, improved patient survival has been reported in smaller series with strictly selected patients . HCC is not observed frequently and has less impact on patient survival following Ltx in PSC patients .
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