Primary biliary Cirrhosis/Cholangitis

Question 1

Define Primary Biliary Cirrhosis

Answer 1

Chronic autoimmune disease of the small intrahepatic bile ducts that is characterised by cholestasis, progressive bile duct damage (+ eventual loss_ due to chronic portal tract inflammation

Question 2

Aetiology of Primary Biliary Cirrhosis

Answer 2

Autoimmune damage to the small bile ducts -> intrahepatic cholestasis -> progressive bile duct damage + eventual loss -> fibrosis -> cirrhosis

Unknown environmental triggers
Genetic predisposition -> loss of immune tolerance to self-mitochondrial proteins

Question 3

Risk factors for Primary Biliary Cirrhosis

Answer 3

Female
Family history 
Autoimmune diseases e.g. T1DM, scleroderma, coeliac 
Age 35-60 
Hypercholesterolaemia 
Recurrent UTIs
Smoking
Pregnancy 
Nail polish/hair dye

Question 4

Symptoms of Primary Biliary Cirrhosis

Answer 4

Often asymptomatic and picked up on LFTs

Itch 
Fatigue, sleep disturbance, daytime somnolence
Dry eyes and mouth 
Postural dizziness/blackouts
Weight loss
Memory and concentration problems

Question 5

Signs of Primary Biliary Cirrhosis on examination

Answer 5

Dry eyes and mouth 
Hepatomegaly 
Jaundice 
Ascites
Splenomegaly 
Skin pigmentation (autoimmune)
Xanthelasma

Question 6

Investigations for Primary Biliary Cirrhosis

Answer 6

LFTs: Raised ALP + GGT, raised ALT and bilirubin
AMA: positive
ANA: staining pattern of multiple dots within the nucleus or along the rim
USS abdomen: exclude obstructive lesions (required)

Serum albumin: reduced
ELISA testing: either indicates presence of AMA or ANA
Clotting studies: PT prolonged
Serum immunoglobulin: polyclonal elevation of IgM and IgG
Serum alpha-fetoprotein: exclude carcinoma
TFTs: TSH raised
Lipid profile: Cholesterol raised

Upper GI endoscopy: exclude varices
Liver biopsy: bile duct lesions, granulomata formation, less bile ducts