Primary Biliary Cirrhosis (PBC)

Exam Cram

• Primary Biliary Cirrhosis (PBC) is an autoimmune condition which targets the extrahepatic biliary ducts

• Presentation: tiredness, itching, xanthelasma, painless jaundice

• Investigations: 1) anti mitochondrial antibody positive, 2) MRCP

• Management: 1) USD, 2) Liver Transplant

Outline

Background

• PBC is an autoimmune condition causing inflammation of the extrahepatic biliary ducts

• Prognosis: survival post diagnosis c.10yrs [1] due to development of liver cirrhosis

• Associated with Lichen Planus 

Assessment

History

• Onset 35-65yrs, 

• Ten times more common in women

• Tiredness, itching, 

Examination

• Xanthelasma, 

• Painless jaundice

Investigations

• Cholestatic LFTs, raised IgM

• Anti mitochondrial antibody positive

• MRCP- extrahepatic duct inflammation

• Liver biopsy

Diagnosis

Diagnostic criteria: (2/3) [2]

1. Elevated alkaline phosphatase

2. Positive anti-mitochondrial antibody

3. Liver histology consistent with PBC

Differentials: Primary Sclerosing Cholangitis

Management

Acute and chronic

1. USD

• •  Doesn't improve outcomes but still recommended for symptomatic relief

2. Liver transplant

• • Definitive treatment

Page written in 2024. 

Resources & References

1. https://pmc.ncbi.nlm.nih.gov/articles/PMC7437077/#:~:text=In%20one%20of%20the%20largest,mortality%20risk%20index%20of%202.9.

2. Bowlus CL, Gershwin ME. The diagnosis of primary biliary cirrhosis. Autoimmun Rev. 2014 Apr-May;13(4-5):441-4. doi: 10.1016/j.autrev.2014.01.041. Epub 2014 Jan 11. PMID: 24424173; PMCID: PMC3987745.

Links

• Body systems: gastrointestinal, haematopoietic & immune systems

• Specialties: Gastroenterology