Primární biliární cholangitida.

Primary biliary cholangitis (PBC) is a chronic autoimmune disorder of the liver. In its long‑term course, it leads to small bile ducts destruction, cholestasis, liver fibrosis, cirrhosis and chronic liver failure. PBC is much common in women, especially of middle‑age, most patients are diagnosed in...

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Bibliographic Details
Published in:Medicina po Promoci Vol. 22; no. 2; pp. 124 - 130
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Format: Article
Published: Medical Tribune CZ, s.r.o., 2021
Online Access:Go to the source
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Summary:Primary biliary cholangitis (PBC) is a chronic autoimmune disorder of the liver. In its long‑term course, it leads to small bile ducts destruction, cholestasis, liver fibrosis, cirrhosis and chronic liver failure. PBC is much common in women, especially of middle‑age, most patients are diagnosed in an asymptomatic stage. The diagnosis is based on the combination of laboratory assessments, alkaline phosphatase elevation more than 1,5 ULN for more than 6 months, and the presence of AMA in a titre 1 : 40 or higher. The typical histological finding confirms the diagnosis, but the stage of liver disease may be determined based on the non‑in‑ vasive liver stiffness measurement. Ursodeoxycholic acid represents nowadays standard‑of‑care in PBC patients, followed by obeticholic acid in non‑responders. Liver transplantation is indicated it those with liver failure in whom conservative therapy failed.