Recurrence of the autoimmune liver disease after liver transplantation

Abstract

Transplantacija jetre uspješna je metoda liječenja bolesnika u terminaloj fazi autoimunih bolesti jetre poput primarne bilijarne ciroze, primarnog sklerozirajućeg kolangitisa i autoimunog hepatitisa. Klinička prezentacija autoimunih bolesti jetre je varijabilna, od akutnog jetrenog zatajenja do postupnog razvoja terminalne bolesti jetre i njezinih komplikacija. Indikacije za transplantaciju kod autoimunih bolesti jetre bitno se ne razlikuju od indikacija kod drugih akutnih i kroničnih jetrenih zatajenja. Usprkos dobrim ishodima nakon transplantacije, ponovna pojava bolesti u donorskoj jetri nije rijetkost, te doseže incidenciju do 40%. Dijagnoza ponavljajuće bolesti postavlja se na temelju kombinacije kliničkih, laboratorijskih i morfoloških parametara što ponekad predstavlja izazov s obzirom na entitete specifične toj populaciji, poput akutnog odbacivanja presatka, virusnih bolesti, ali i de novo autoimunog hepatitisa. Važnost prepoznavanja ponavljajuće autoimune bolesti jetre u presatku klinički je značajna budući da utječe na pristup kako imunosupresivnog tako i neimunosupresivnog liječenja čime se sprečava ili usporava progresija bolesti i smanjuju potencijalne komplikacije. Svrha ovog preglednog rada je prikazati dosadašnja saznanja o autoimunim bolestima jetre u kontekstu transplantacije, s osvrtom na incidenciju, rizične čimbenike, dijagnostičke kriterije i terapijske opcije, te prognozu bolesnika nakon transplantacije.Liver transplantation is an effective treatment for patients with end-stage autoimmune liver diseases such as primary biliary cirrhosis, primary sclerosing cholangitis and autoimmune hepatitis. Clinical presentation of autoimmune liver diseases varies from acute liver failure to progressive development of end-stage liver disease and its complications. Indications for liver transplantation in autoimmune liver disease do not differ substantially from those used for other acute or chronic liver diseases. Despite the good reported outcomes, the recurrence of autoimmune liver disease is relatively common after liver transplantation, with incidence reaching up to 40%. The combination of clinical, laboratory and morphological tests are required, to establish the diagnosis of recurrent autoimmune diseases, however it may represent a challenge due to specific entities in transplant population such as acute graft rejection, viral hepatitis and even de novo autoimmune hepatitis. An awareness of the existence of recurrent autoimmune liver diseases in the allograft has important clinical implications on both immunosuppressive or non-immunosppressive treatment approaches, preventing or decelerating disease progression and reducing potential complications. The aim of this paper is to review the present knowledge of autoimmune liver diseases within the context of the transplantation, overviewing the incidence, risk factors, diagnostic criteria and treatement options, as well as outcomes for patients after transplantation