Diagnosis and treatment of primary sclerosing cholangitis

Primarni sklerozirajući kolangitis (PSC) kronična je, imunosno posredovana bolest jetre. Karakterizira je kronična upala i fibroza intra- i ekstrahepatičnih žučnih vodova. Kroz 10 do 15 godina, bolest u većine bolesnika dovodi do dekompenzirane ciroze jetre zbog kolestaze i progresivne jetrene fibroze. Incidencija PSC-a je 1 na 100 000 stanovnika. Procijenjeno je da 60% do 80% bolesnika s PSC-om boluje i od upalne bolesti crijeva. Etiologija bolesti nejasna je. U trenutku postavljanja dijagnoze većina bolesnika je asimptomatska te na bolest upućuju abnormalni testovi jetrene funkcije i/ili otkriće proširenja žučnih vodova na radiološkim snimkama. Od simptomatskih bolesnika, umor i svrbež najčešći su simptomi u početku bolesti. S napredovanjem bolesti javljaju se i žutica, gubitak težine i abdominalna bol. Zlatni standard u dijagnozi PSC-a je kolangiopankreatografija magnetskom rezonancijom (MRCP) koja je zamijenila endoskopsku retrogradnu kolangiopankreatografiju (ERCP). Kod liječenja PSC-a koristi se ursodeoksikolna kiselina, intervencije ERCP-om i transplantacija jetre. PSC sporo je progresivna bolest i većina bolesnika umre u roku od 10 do 12 godina od postavljanja dijagnoze, ako im se ne transplantira jetra. Značajan broj bolesnika oboli od hepatobilijarnog ili kolorektalnog karcinoma.Primary sclerosing cholangitis (PSC) is a chronic, immune-mediated liver disease. It is characterised by chronic inflammation and fibrosis of intra- and extrahepatic bile ducts. In the course of 10 to 15 years, in most patients the disease leads to decompensated liver cirrhosis due to cholestasis and progressive liver fibrosis. The incidence of PSC is 1 per 100, 000 population.It is estimated that 60 to 80 % patients with PSC have inflammatory bowel disease.The etiology of the disease is unclear. At the time of diagnosis, most patients are asymptomatic and only abnormal liver tests and/or the discovery of dilated bile ducts on imaging findings indicate that the disease is present. In symptomatic patients, fatigue and pruritus are the most common symptoms at disease onset. As the disease advances, jaundice, weight loss and abdominal pain occur. The gold standard in diagnosing PSC is magnetic resonance cholangiopancreatography (MRCP), which replaced endoscopic retrograde cholangiopancreatography (ERCP). PSC is treated with ursodeoxycholic acid, ERCP interventions and liver transplant. PSC is a slowly progressive disease and most patients die within 10 to 12 years from the time when the disease was diagnosed, if they do not recieve a liver transplant. A significant number of patients develop hepatobiliary or colorectal cancer