EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis | 2017 EASL

Doenças Colestáticas Autoimunes 2017

Summary
Primary biliary cholangitis (PBC) is a chronic inflammatory
autoimmune cholestatic liver disease, which when untreated will
culminate in end-stage biliary cirrhosis. Diagnosis is usually
based on the presence of serum liver tests indicative of a cholestatic hepatitis in association with circulating antimitochondrial
antibodies. Patient presentation and course can be diverse and
risk stratification is important to ensure all patients receive a personalised approach to their care. The goals of treatment and management are the prevention of end-stage liver disease, and the
amelioration of associated symptoms. Pharmacologic approaches
in practice, to reduce the impact of the progressive nature of
disease, currently include licensed therapies (ursodeoxycholic
acid and obeticholic acid) and off-label therapies (fibric acid
derivatives, budesonide). These clinical practice guidelines
summarise the evidence for the importance of a structured,
life-long and individualised, approach to the care of patients with
PBC, providing a framework to help clinicians diagnose and
effectively manage patients.
 2017 European Association for the Study of the Liver. Published
by Elsevier B.V. All rights reserved.

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