Primary Biliary Cholangitis Flashcards
What is PBC?
Primary biliary cholangitis
A chronic liver disorder
What is the pathophysiology of PBC?
Interlobular bile ducts are damaged by chronic granulomatous inflammation which causes progressive cholestasis which leads to cirrhosis
What conditions are associated with PBC?
Thyroid disease
RA
Sjogren’s syndrome
Systemic sclerosis
What are the risk factors for PBC?
Female
40-60 years
FHx of autoimmune disease
Personal history of autoimmune disease
What are the features of PBC?
Often asymptomatic
Fatigue Pruritus Jaundice Hyperpigmentation Arthralgia
What investigations are done in suspected PBC?
Bloods
- LFTs
- clotting screen
- TFTs
- lipids
AMA antibodies
USS of liver
What do the LFTs show in PBC?
Raised ALP
Raised ALT
Raised GGT
How is the pruritus in PBC managed?
Chostyramine
How is PBC managed medically?
Cholestryamine
Fat-soluble vitamin supplements
Ursodeoxycholic acid
What is surgical management of PBC?
Liver transplant
When is liver transplant indicated in PBC?
Bilirubin >100
What are the complications of PBC?
Cirrhosis
Osteomalacia/osteoporosis
Increased risk of HCC
