Primary Biliary Cholangitis (PBC) Flashcards
What does PBC stand for?
Primary Biliary Cholangitis
What is Primary Biliary Cholangitis?
Intrahepatic autoimmune jaundice affecting intralobular bile ducts (not common bile duct)
What are risk factors for PBC?
Female
40-50 y/o
Other autoimmune disease
Smoking
Is Primary Biliary Cholangitis or Primary Sclerosing Cholangitis more common?
Primary Biliary Cholangitis
In PBC, autoantibodies cause intralobular bile duct damage causing c____ a_______ g_______ i________
chronic autoimmune granulomatous inflammation
The chronic autoimmune granulomatous inflammation of the intrahepatic bile ducts (in PBC) results in…
Cholestasis
Fibrosis and Cirrhosis
Portal hypertension
Infection
How is PBC commonly identified in asymptomatic patients?
Routine test shows increased AMA (antimitochondrial antibodies)
What are symptoms of PBC?
Pruritis (conjugated bilirubin excess leads out and causes skin to itch)
Then jaundice
Then hepatomegaly and xanthelasma
What are complications of PBC?
Cirrhosis
Malabsorption of fats and vitamins A,D,E and K
Steatorrhoea
Osteomalacia
Coagulopathy
How is PBC diagnosed?
LFT:
Elevated ALP and conjugated bilirubin
Lowered levels albumin
Rule out Acute hepatitis:
HepBsAg and HCVAb
Serology:
95% have AMA antibodies
1st line imaging to exclude extrahepatic cholestasis:
USS
Liver biopsy
What would a liver biopsy show in a patient with PBC?
Portal tract infiltrate (lymphocytes and plasma cells)
40% granulomatous
Portal tract fibrosis
What is 1st line treatment for PBC?
Lifelong Ursodeoxycholic acid
(Bile acid analogue, dampens immune response and decreases cholestasis)
What is treatment for pruritis in PBC?
Colestyramine
What other treatment should be considered for PBC alongside ursdeoxycholic acid and colestyramine?
Vitamin ADEK supplements
(consider osteomalacia)
May ultimately need liver transplant
