Recurrence of primary sclerosing cholangitis in the graft

Abstract

Primary sclerosing cholangitis (PSC) is a disease characterized by inflammation, fibrosis and obliteration of both intra- and extrahepatic bile ducts, accompanied by cholestasis, with further outcome in biliary cirrhosis of the liver, cholangiocarcinoma. The pathogenesis of the disease is poorly understood, but, according to various sources, it involves genetic factors, innate and adaptive immunity mechanisms, the toxic effects of hydrophobic bile acids and, possibly, intestinal dysbiosis. The strong association with inflammatory bowel disease is associated with a significantly increased risk of colorectal cancer, which, along with cholangiocarcinoma, represents the most significant diagnostic challenge in the long-term management of PSC. The diagnosis of PSC is established based on the identification of typical cholangiographic lesions of the bile ducts and the exclusion of secondary causes of sclerosing cholangitis. Complex pathophysiology, heterogeneity of clinical features and the rare nature of the disease have led to the lack of effective therapy to date; there are no treatment algorithms, but a course of ursodeoxycholic acid in doses of 17–23 mg/kg/day can be prescribed for up to a year in order to monitor the dynamics of the decrease in levels serum alkaline phosphatase. A number of drugs are under investigation, including FXR (farnesoid X receptor) agonists with choleretic and antimicrobial properties. Clinically significant stenoses can be successfully treated with interventional endoscopy, but liver transplantation (LT) is currently the only curative treatment with a high survival rate. According to various literature data, 20–25% of patients develop disease relapse in the graft. Our case report of recurrent PSC in a patient 5 years after orthotopic LT provides an overview of management options from a practical, patient-centered perspective.