Primary sclerosing cholangitis

Primarni sklerozirajući kolangitis (PSK) kronična je kolestatska bolest jetre nepoznate etiologije karakterizirana progresivnom upalom i fibrozom koja vodi do multifokalnih striktura intrahepatalnih i ekstrahepatalnih žučnih vodova. Bolest obično polagano napreduje kroz period od 10 do 15 godina razvitkom ciroze jetre i njezinim komplikacijama portalnom hipertenzijom i u konačnici zatajenjem jetrenih funkcija. Epidemiologija PSK u općoj populaciji još uvijek nije točno utvrđena. Oko 70% oboljelih čine muškarci, a prosječna dob pri dijagnozi je 40 godina. U otprilike 75% slučajeva udružena je s crijevnom upalnom bolešću. Smatra se da je bolest multifaktorska te da je za njen razvitak potrebno djelovanje okolišnih čimbenika u genetski predisponiranih pojedinaca koji pokretanjem autoimunih procesa dovode do oštećenja kolangiocita i progresivne fibroze. Komplikacije bolesti uključuju kolelitijazu, koledoholitijazu, bilijarne strikture, kolangiokarcinom, hepatocelularni karcinom, kolorektalni karcinom, pogotovo u pacijenata s ulceroznim kolitisom. Zlatni je standard za dijagnozu PSK endoskopska retrogradna kolangiopankreatografija, a tipičan nalaz predstavljaju multifokalna segmentalna suženja s dilatiranim vodovima koji daju izgled krunice. Zasada ne postoji medikamentna terapija za koju je dokazano da usporava progresiju bolesti, ali antibiotici i monoklonalno protutijelo s antifibrotičkim učinkom daju obećavajuće rezultate. U terminalnom stadiju bolesti učinkovita je transplantacija jetre.Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology, characterised by progressive inflammation and fibrosis which results in multifocal intrahepatic and extrahepatic biliary strictures. The disease usually develops slowly during a period of 10 to 15 years by developing liver cirrhosis, which causes portal hypertension and, ultimately, liver failure. The epidemiology of PSC in the general population has not been precisely determined. Approximately 70% of patients are men, with a mean age at diagnosis of 40. In approximately 75% of cases, the patients also have inflammatory bowel disease. It is considered that the disease is multifactorial and that its development is triggered by environmental factors in people with a genetic predisposition which via autoimmune processes lead to cholangiocyte damage and progressive fibrosis. Complications of the disease include cholelithiasis, choledocholithiasis, biliary strictures, cholangiocarcinoma, hepatocellular carcinoma, colorectal carcinoma, especially in patients with ulcerative colitis. The gold standard for the diagnosis of PSC is endoscopic retrograde cholangiopancreatography, and a typical finding consists of multifocal segmental strictures with dilated bile ducts that have a beaded appearance. At this moment, a medicament therapy proven to slow down the progression of the disease does not exist, but antibiotics and monoclonal antibody with antifibrotic effect show promising results. Liver transplantation is effective at the terminal stage of the disease