The relative proportion of patients with primary sclerosing cholangitis (PSC) transplanted among patients with autoimmune liver disease is increasing, that of primary biliary cirrhosis (PBC) has had a decrease and subsequent increase, whereas the relative proportion of autoimmune hepatitis (AIH) patients is stable. The reason for this increase among PSC-patients has previously not been investigated. We did a retrospective investigation of 222 patient charts to identify the cause for transplantation, and found that 50 (36,0%) PSC-patients were transplanted because of symptoms of liver failure, 61 (43,8%) because of subjective symptoms (pruritus, icterus and multiple episodes of cholangitis) and 25 (18%) because of suspicion of malignancy. Among PBC-patients 36 (62,1%) were transplanted because of liver failure and 21 (36,2%) because of subjective symptoms. Among AIH-patients, 25 (100%) were transplanted because of liver failure. The number of PSC-patients that had previously suffered from encephalopathy, variceal bleeding or ascites decreased over the course of the study. Their biochemical profile improved, with increasing hemoglobin, thrombocytes and albumin and decreasing towards normalization of INR, AST, ALT and bilirubin. We found no corresponding improvement in clinical and biochemical parameters in PBC- and AIH-patients except for a slight improvement of biochemical profile in PBC-patients . These findings suggest that PSC-patients are now listed for liver transplantation at an earlier stage in the disease progression. Survival analysis shows that PSC- and PBC-patients transplanted because of liver failure has a 10% lower 5 year survival rate than the patients with the same diagnosis transplanted because of subjective symptoms. PSC-patients transplanted because of suspicion of malignancy has 90,5% 5 year survival.