Primary Sclerosing Cholangitis: A Clinical Case

The basic hypotheses of pathogenesis of primary sclerosing cholangitis (PSC) are discussed, i.e. genetically conditioned pathology, autoimmune pathology, the result of inflammatory reaction in bile ducts, and cholangiopathy. A clinical case of monozygotic twins with association of PSC and non-specific ulcerative colitis (NUC) is presented. The first twin had a severe course of PSC and a mild course of NUC; he died due to bacterial complications of cholangitis. The second twin, patient B, had an opposite situation, a severe course of NUC, while PSC was suspected only after determination of cholestasis biochemical markers. As soon as cholestasis was revealed, patient B was administered Ursofalk and Budenofalk (in 2001). He received Salofalk as a basic therapy for NUC. Repeated liver biopsy in 2005 showed no progression of PSC, but minimal biochemical signs of cholestasis were present in 2009. It is therefore necessary to study the first-degree relatives of patients with PSC.