Liver transplantation for primary sclerosing cholangitis

Abstract

Primary sclerosing cholangitis (PSC) is a chronic, progressive cholestatic liver disease of unknown origin. PSC is closely associated with inflammatory bowel disease, mainly ulcerative colitis (UC). PSC presents a significantly increased risk of hepatobiliary and colorectal neoplastic changes. PSC gradually leads to cirrhosis with a significant portal hypertension. No effective medical therapy is available and treatment with a high dosage of ursodeoxycholic acid could even lead to a number of adverse effects. Liver transplantation (OLTx) is the only curative option currently available to improve survival. In this study, a retrospective analysis was performed for all patients who underwent OLTx for PSC over a 20-year period in the Institute for Clinical and Experimental Medicine (IKEM), Prague.

Results: A total of 102 patients underwent OLTx for PSC with a mean age of 37 years (14-68). Inflammatory bowel disease (IBD) was confirmed in 64 patients (62.8%) pre-LT Except for three cases with Crohn's disease, all patients suffered from UC. Cumulated 1-, 5-, 10- and 15-year patient survival rates were 94.1%, 93.0%, 93.0% and 82.3% respectively. Recurrent PSC was determined in 28 patients (33.7%) with three who needed retransplantation. In six patients, cholangiocarcinoma was incidentally detected in explanted livers and three died due to advanced carcinoma. The course of ulcerative colitis (UC) was mild or in remission in 34/61 (55.7%) patients followed in our centre after OLTx without relevant clinical symptoms. The remaining 27 patients (44.3%) suffered from clinically active disease. Colectomy was performed in four patients due to refractory disease. On the other hand, UC newly developed in 13 patients after OLTx with a mild course of disease.

Conclusion: The outcome for patients with PSC who have undergone transplantation is excellent. PSC frequently recurs in the hepatic allograft but retransplantation is seldom necessary. The course of UC after OLTx for PSC is frequently active despite immunosuppressive treatment. Detection of dysplastic changes and CRC confirm the usefulness of regular colonoscopic evaluations. New onset of UC can develop after OLTx. Incidentally found cholangiocarcinoma remains a difficult treatment problem with a low survival rate.