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

Czech version

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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