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13-09-2022 | Liver Transplantation | Editorial

Secondary Treatment of Primary Biliary Cholangitis: Early Prediction of Inadequate Response to Ursodeoxycholic Acid in Patients with PBC

Authors: Benjamin D. Liu, Kamran Qureshi

Published in: Digestive Diseases and Sciences | Issue 2/2023

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Excerpt

Primary biliary cholangitis (PBC) is an autoimmune disease of the liver with a slowly progressive course that may extend over decades. Since it frequently leads to cirrhosis and liver failure [1], untreated PBC is a common indication for liver transplantation (LT) Histologically, PBC is characterized by inflammatory T-cell-mediated destruction of intrahepatic bile ducts, leading to cholestasis. Clinically, it is identified as a chronically elevated hepatic alkaline phosphatase (ALP). It usually first manifests in females in their fifth or sixth decades of life, who possibly have a genetic predisposition and autoimmune injury that is initiated by environmental triggers [2]. Early diagnosis is challenging since many patients are asymptomatic; the diagnosis is frequently made incidentally by routine laboratory testing [3]. Symptomatic patients may have fatigue, pruritus, bone disease, concurrent autoimmune diseases, dyslipidemia, or features of advanced liver disease [3]. Though elevated ALP is a hallmark of liver injury related to PBC, elevated total bilirubin (TBili), and low albumin (Alb) signal the presence of advanced liver disease and hepatic synthetic dysfunction. Preventing the progression of histologic stages prolongs patient survival [3]. As a result, it is essential to identify and treat PBC early in its course, prior to the development of cirrhosis. Since the Mayo PBC score is a prognostic risk model that predicts survival and need for LT for up to 7 years in untreated patients, it is used to identify high-risk patients and the need for urgent treatment [4]. …
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Metadata
Title
Secondary Treatment of Primary Biliary Cholangitis: Early Prediction of Inadequate Response to Ursodeoxycholic Acid in Patients with PBC
Authors
Benjamin D. Liu
Kamran Qureshi
Publication date
13-09-2022
Publisher
Springer US
Published in
Digestive Diseases and Sciences / Issue 2/2023
Print ISSN: 0163-2116
Electronic ISSN: 1573-2568
DOI
https://doi.org/10.1007/s10620-022-07661-y

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