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Guidelines of the Czech Society of Hepatology for diagnosis and treatment of primary biliary cholangitis


Authors: T. Fejfar 1;  T. Vaňásek 1;  P. Hůlek 1,2;  L. Vítek 3,4;  S. Fraňková 5;  R. Brůha 4
Authors‘ workplace: II. interní gastroenterologická klinika LF UK a FN Hradec Králové 1;  Katedra interních oborů, LF OU a FNsP Ostrava 2;  Ústav lékařské biochemie a laboratorní diagnostiky, 1. LF UK a VFN v Praze 3;  IV. interní klinika – klinika gastroenterologie a hepatologie 1. LF UK a VFN v Praze 4;  Klinika hepatogastroenterologie, Transplantcentrum, IKEM, Praha 5
Published in: Gastroent Hepatol 2018; 72(2): 109-118
Category: Hepatology: Best Practises
doi: https://doi.org/10.14735/amgh2018109

Primární biliární cholangitida je chronické autoimunní cholestatické onemocnění. Diagnóza je založena na nálezu cholestázy, průkazu specifických antimitochondriálních protilátek nebo typickém histologickém nálezu. Velká část nemocných je v době diagnózy asymptomatická. Pokud jsou symptomy přítomny, nejčastěji se objevuje pruritus a únava. Základní léčbou 1. linie je ursodeoxycholová kyselina. U nemocných, kteří na léčbu ursodeoxycholovou kyselinou neodpoví adekvátním ústupem cholestázy, zůstává prognóza nepříznivá. Pro tyto nemocné lze zvážit podání léčby 2. linie. Léčbu vyžadují i často přidružené symptomy nebo komplikace, jako je pruritus a kostní choroba. V pokročilých stadiích vyžadují léčbu komplikace asociované portální hypertenze. Ve stadiu jaterní cirhózy je nutno zvážit transplantaci jater.

Overview

Primary biliary cholangitis is a chronic cholestatic disease that is diagnosed based on cholestatic laboratory features, antimitochondrial antibody positivity, or a typical histological pattern. Most patients are asymptomatic at the time of diagnosis. Pruritus and fatigue are the most common symptoms in symptomatic patients. Ursodeoxycholic acid is the first-line therapy. If this fails, the prognosis is unfavorable and second-line therapy can be considered. The associated symptoms must be treated. In advanced disease, liver transplantation should be considered and the complication of portal hypertension must be treated.

Key words:
biliary cholangitis – primary – diagnosis – treatment

The Editorial Board declares that the man­uscript met the ICMJE „uniform requirements“ for bio­­­­medical papers.

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.


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