Author/Editor		Pernat, Cvetka; Skalicky, Marjan
Title		Primarna biliarna ciroza in primarni sklerozantni holangitis
Translated title		Primary biliary cirrhosis and primary sclerosing cholangitis
Type		članek
Source		In: Krajnc I, Pahor A, Skok P, et al, editors. Zbornik predavanj in praktikum 22. srečanje internistov in zdravnikov družinske medicine Iz prakse za prakso; 2011 maj 20-21; Maribor. Maribor: Univerzitetni klinični center,
Publication year		2011
Volume		str. 185-94
Language		slo
Abstract		Primary biliary cirrhosis is a chronic autoimmune cholestatic liver disease of unknown etiology. The most important symptoms include fatigue, pruritus and associated autoimmune symptoms. The diagnosis is based on three criteria: elevation of cholestatic enzymes, the presence of antimitochondrial antibodies in serum and histologic findings. If untreated, disease progresses to cirrhosis and liver failure. Therapy consists of ursodeoxycholic acid; liver transplantation is the final therapeutic option for patient with end-stage liver disease. Primary sclerosing cholangitis is a chronic immune mediated cholestatic liver disease of unknown etiology, often associated with inflammatory bowel disease. Many patients are asymptomatic at diagnosis or have mild symptoms, such as fatigue and pruritus. In most the disease progress to cirrhosis and liver failure. Cholangiocarcinoma develops in 8 to 30% of patients. The diagnosis is based on typical cholangiographic and histological findings. High dose ursodeoxycholic acid may have beneficial treatment effect. Liver transplantation is the final therapeutic option for patient with end-stage liver disease.
Summary		Primarna biliarna ciroza je kronična avtoimuna holestatska jetrna bolezen. Glavni znaki bolezni so utrujenost, srbečica in znaki drugih avtoimunih bolezni. Diagnoza temelji na treh kriterijih: povišane vrednosti holestatskih encimov, prisotnost antimitohondrijskih protiteles v serumu in histološke spremembe. Če bolezni ne zdravimo, napreduje v jetrno cirozo. Zdravimo jo z ursodeoxiholno kislino. Pri bolnikih z izraženo cirozo in jetrno odpovedjo je končna oblika zdravljenja presaditev jeter. Primarni sklerozantni holangitis je kronična, imunsko pogojena holestatska bolezen jeter, pogosto pridružena kronični vnetni črevesni bolezni. Mnogo bolnikov je ob postavitvi diagnoze brez težav ali opisujejo le blago izraženo utrujenost in srbečico. Večinoma bolezen napreduje v cirozo z jetrno odpovedjo. Pri 8 do 30% bolnikov se razvije holangiokarcinom. Diagnoza temelji na tipičnem holangiogramu in histoloških spremembah. V zdravljenju so učinkoviti visoki odmerki ursodeoksiholne kisline. Pri bolnikih z izraženo cirozo in jetrno odpovedjo je končna oblika zdravljenja presaditev jeter.
Descriptors		LIVER CIRRHOSIS, BILIARY CHOLANGITIS, SCLEROSING