Primary Biliary Cholangitis (Primary Biliary Cirrhosis) Questions & Answers

Updated: Nov 08, 2017
  • Author: Nikolaos T Pyrsopoulos, MD, PhD, MBA, FACP, AGAF, FAASLD, FRCP(Edin); Chief Editor: BS Anand, MD  more...
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Questions & Answers

Overview

What is primary biliary cholangitis (PBC)?

What are the symptoms of primary biliary cholangitis (PBC)?

Which physical findings are characteristic of primary biliary cholangitis (PBC)?

What are physical findings suggestive of advanced primary biliary cholangitis (PBC)?

Which lab abnormalities are characteristic of primary biliary cholangitis (PBC)?

Which lab test findings are less common indicators of primary biliary cholangitis (PBC)?

What is the role of antimitochondrial antibodies (AMAs) in the diagnosis of primary biliary cholangitis (PBC)?

What is the role of imaging studies in the diagnosis of primary biliary cholangitis (PBC)?

Which findings of portal hypertension may be present in primary biliary cholangitis (PBC)?

What is the staging for primary biliary cholangitis (PBC)?

What are the pharmacologic treatment options for primary biliary cholangitis (PBC)?

How is pruritus managed in primary biliary cholangitis (PBC)?

What is primary biliary cholangitis (PBC)?

What is the historical background of primary biliary cholangitis (PBC)?

Which patients are at highest risk for primary biliary cholangitis (PBC)?

What is the pathophysiology of primary biliary cholangitis (PBC)?

What is the role of chronic cholestasis in the pathogenesis of primary biliary cholangitis (PBC)?

Which factors may induce primary biliary cholangitis (PBC)?

What is the role of genetics in the etiology of primary biliary cholangitis (PBC)?

What is the prevalence of primary biliary cholangitis (PBC) in the US?

What is the global prevalence of primary biliary cholangitis (PBC)?

What are the racial predilections of primary biliary cholangitis (PBC)?

How does the prevalence of primary biliary cholangitis (PBC) vary by sex?

How does the prevalence of primary biliary cholangitis (PBC) vary by age?

How does fatigue level affect the prognosis of primary biliary cholangitis (PBC)?

What determines the prognosis of primary biliary cholangitis (PBC)?

What is the mortality rate for primary biliary cholangitis (PBC)?

What are the possible complications of primary biliary cholangitis (PBC)?

Presentation

Which history findings are characteristic of primary biliary cholangitis (PBC)?

What is the prevalence of pruritus in primary biliary cholangitis (PBC)?

What is prevalence of right upper quadrant discomfort in primary biliary cholangitis (PBC)?

Which physical findings are characteristic of primary biliary cholangitis (PBC)?

DDX

What are diagnostic considerations for primary biliary cholangitis (PBC)?

What are the differential diagnoses for Primary Biliary Cholangitis (Primary Biliary Cirrhosis)?

Workup

What is the role of lab studies in the diagnosis of primary biliary cholangitis (PBC)?

What is the hallmark lab finding of primary biliary cholangitis (PBC)?

What is the role of imaging studies in the workup of primary biliary cholangitis (PBC)?

Which procedure is performed to confirm a diagnosis of primary biliary cholangitis (PBC)?

Which histologic findings are characteristic of primary biliary cholangitis (PBC)?

How is primary biliary cholangitis (PBC) staged?

Treatment

What are the goals of treatment for primary biliary cholangitis (PBC)?

What is the role of ursodeoxycholic acid (UDCA) in the treatment of primary biliary cholangitis (PBC)?

What is the role of obeticholic acid in the treatment of primary biliary cholangitis (PBC)?

Which medications are used in the treatment of primary biliary cholangitis (PBC)?

How is pruritus managed in primary biliary cholangitis (PBC)?

Which activity recommendations are beneficial in the treatment of primary biliary cholangitis (PBC)?

What is the role of surgery in the treatment of primary biliary cholangitis (PBC)?

Medications

What are the goals of treatment for primary biliary cholangitis (PBC)?

Which medications in the drug class Antihistamines are used in the treatment of Primary Biliary Cholangitis (Primary Biliary Cirrhosis)?

Which medications in the drug class Antipruritics are used in the treatment of Primary Biliary Cholangitis (Primary Biliary Cirrhosis)?

Which medications in the drug class Anti-inflammatory agents are used in the treatment of Primary Biliary Cholangitis (Primary Biliary Cirrhosis)?

Which medications in the drug class Immunosuppressants are used in the treatment of Primary Biliary Cholangitis (Primary Biliary Cirrhosis)?

Which medications in the drug class Farnesoid X Receptor Agonist are used in the treatment of Primary Biliary Cholangitis (Primary Biliary Cirrhosis)?

Which medications in the drug class Bile acids are used in the treatment of Primary Biliary Cholangitis (Primary Biliary Cirrhosis)?