Autoimmune Liver Diseases

Autoimune bolesti jetre uključuju autoimuni hepatitis (AIH), primarni bilijarni kolangitis (PBC) i primarni sklerozirajući kolangitis (PSC), te se nerijetko pojavljuju i zajedno u vidu sindroma preklapanja (overlap). Dok AIH primarno pogađa jetrene stanice, druge dvije bolesti zahvaćaju bilijarni epitel i tipično se prezentiraju svrbežom, umorom, žuticom te kolestatskim profilom u laboratorijskim nalazima. AIH i PBC pogađaju dominantno osobe ženskog spola i imaju specifične, gotovo patognomonične serološke biljege, dok se PSC tipično javlja kod mladih muškaraca oboljelih od upalnih bolesti crijeva. Ove bolesti pretežito su asimptomatske ili vrlo nespecifičnih simptoma, zbog čega često prođu nezapaženo i otkriju se tek u stadiju uznapredovane fibroze ili ciroze. Dok je biopsija jetre zlatni standard u dijagnostici AIH i sindroma preklapanja, MRCP je metoda izbora u PSC-u. U terapijskom smislu, AIH uglavnom pokazuje dobar odgovor na imunosupresivnu terapiju za koju je ključno ne prekinuti je do postizanja ne samo biokemijske, već i histološke remisije, što nužno zahtijeva barem tri godine liječenja. Ursodeoksikolna i obetikolna kiselina te ERCP kod striktura ekstrahepatalnih žučnih vodova zasad su jedini modaliteti liječenja kolestatskih bolesti jetre. U razdoblju dekompenzirane ciroze jetre transplantacija ostaje kao posljednja mogućnost izlječenja, osobito u slučaju razvoja hepatocelularnog karcinoma.Autoimmune liver diseases include autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Sometimes they occur simultaneously in the form of the ‘overlap syndrome’. While AIH primarily affects liver cells, the other two diseases affect biliary epithelium and typically present with pruritus, fatigue, jaundice, and cholestatic profile in laboratory findings. AIH and PBC affect predominantly females and have specific, almost pathognomonic serologic features, whereas PSC typically occurs in young men with inflammatory bowel disease. These diseases are mostly asymptomatic, or manifest themselves in nonspecific symptoms, and are therefore often detected in advanced stages of fibrosis or cirrhosis. While liver biopsy is the gold standard in the diagnosis of AIH and the overlap syndrome, MRCP is the method of choice for PSC. Regarding therapy, AIH generally responds well to immunosuppressive therapy, but requires continued treatment until biochemical and histological remission is achieved, which inevitably implies at least three years of treatment. Ursodeoxycholic acid, obeticholic acid, and ERCP in extrahepatic bile duct strictures are currently the only modalities for the treatment of cholestatic liver diseases. In decompensated liver cirrhosis or hepatocellular cancer, liver transplantation remains the last treatment option