Dominykas Stankevičius, Raimundas Darafėjus

Faculty of Medicine, Vilnius University

Abstract

Primary sclerosing cholangitis is a chronic, unknown etiology intra- and/or extrahepatic bile ducts disease causing the inflammation and fibrosis of them. As the disease progresses, cirrhosis of the liver or cholangiocarcinoma may develop, thus more than 50% of the patients require a liver transplant within the first 10 to 15 years after the first symptoms occur. Primary sclerosing cholangitis affects men more often, and their average age at diagnosis ranges from 30 to 40 years. The exact etiology of this disease is still unknown, but a clear association with inflammatory bowel disease has been established. Up to 80% of patients with primary sclerosing cholangitis are also diagnosed with inflammatory bowel disease (ulcerative colitis or Crohn‘s disease). The literature presents various hypotheses that investigate the pathogenetic link between these diseases: the influence of intestinal wall permeability, microbiota, bile acids, cytokines and genes on the development of the disease is considered. Magnetic resonance cholangiopancreatography remains the most accurate diagnostic test, but endoscopic retrograde cholangiopancreatography is also often used. There is no effective medication treatment yet. Although, the positive effects of biological therapy are observed, but further studies are needed. Colectomy may be an effective surgical solution in the treatment of inflammatory bowel disease and in inhibition of primary sclerosing cholangitis course. Liver transplantation is required as the disease progresses, and cirrhosis or cholangiocarcinoma develops. However, if the course of inflammatory bowel disease is uncontrolled, the recurrence of primary sclerosing cholangitis may develop even in the transplanted liver.

Keywords: primary sclerosing cholangitis, inflammatory bowel disease, ulcerative colitis, Crohn’s disease.