Primary Biliary cirrhosis

Question 1

What is primary biliary cirrhosis?

Answer 1

Primary biliary cholangitis (PBC) is a chronic disease of the small intrahepatic bile ducts that is characterised by progressive bile duct damage (and eventual loss) occurring in the context of chronic portal tract inflammation.

Question 2

What are the risk factors of primary biliary cirrhosis?

Answer 2

> Female
> 45-60
> Smoking
> UTI
>FHx

Question 3

What is the epidemiology of primary biliary cirrhosis?

Answer 3

The prevalence of primary biliary cholangitis (PBC) is up to 35/100,000 in US populations, with a distribution that is heavily skewed towards women (10:1 female-to-male distribution) and those over 45 years of age.

Question 4

What are the signs and symptoms of primary biliary cirrhosis?

Answer 4

> Itch
> Fatigue
> Dry eyes and mouth
> Sleep disturbance
> Postural dizziness/ blackouts
> Hepatomegaly
> Weight loss and other metabolic features of mal-absorption
> Memory/ Conc problems
> Jaundice
> Ascites
> Splenomegaly
> Skin pigmentation
> Xanthelasmata

Question 5

What investigations would you do for primary biliary cirrhosis?

Answer 5

Bloods
Abdo US
MRCP
Upper GI endoscopy
Liver biopsy

Question 6

What bloods would you do for someone with primary biliary cirrhosis?

Answer 6

> Alk Phos
> GGT
> BR
> ALT
> Serum albumin
> Anti-mitochondrial antibody immunofluorescence
> ANA
> Anti- pyruvate dehydrogenase complex E2-ELISA
> Anti M2 ELISA
Anti-glycoprotein-210 ELISA
> Anti Sp100 ELISA