Aleksandr Marchockij¹, Dominyka Stragytė¹, Vitalija Petrenkienė²

¹Lithuanian University of Health Sciences, Faculty of Medicine²Lithuanian University of Health Sciences, rare digestive system and liver disease center

 ^ABSTRACT

Primary sclerosing cholangitis (PSC) is a rare progressive liver disease, which ultimately leads to cirrhosis and liver failure. Patients are often asymptomatic and the disease is diagnosed accidentally after investigating the cause of elevated liver enzymes. The prevalence of PSC is approximately 1-10/100,000. A peak incidence of the diagnosis is around 40 years, while male preponderance is around two times higher than female. PSC is closely related to inflammatory bowel disease (IBD), especially ulcerative colitis (UC). The most important diagnostic method is magnetic resonance cholangiopancreatography (MRCP). The typical cholangiographic findings include focal stricturing and saccular dilatation of the bile ducts, which may lead to a “beaded” appearance. At this time, there is no established medical treatment, which could slow the progression of PSC. However, in those with advanced disease liver transplantation results in good long-term outcomes.

Keywords: Primary sclerosing cholangitis, Magnetic Resonance Cholangiopancreatography (MRCP), ulcerative colitis, ursodeoxycholic acid.