Biliary Disease

Question 1

Where in the biliary tract does Primary biliary cirrhosis occur.

Answer 1

Small intrahepatic ductules

Question 2

Two most common symptoms of PBC and diagnosis.

Answer 2

1. Fatigue
2. Pruritus (itching)

Dx: increased GGTP, ALP, cholesterol, IgM

Question 3

Describe the 4 stages of PBC.

Answer 3

1. Lymphoplasmocytic inflammation of the portal tract, destruction of septal and intralobular bile ducts.
2. periportal extension of stage 2
3. Cholestasis and loss of many of the intralobular ducts
4. biliary cirrhosis

Question 4

What other proteins might rise in the serum in a patient with PBC?

Answer 4

ANA, ASMA, rheumatoid factor, anti-thyroid antibodes.
(associated with other autoimmune disease)

Anti-mitochondrial antibody (similar to E. Coli epitope)

Question 5

Main complication of PBC.

Answer 5

Osteoporosis

Question 6

Treatment of PBC.

Answer 6

UDCA

Vit. D (if progression to osteoporosis)

Question 7

What differentiates PBC from autoimmune cholangitis in serum testing.

Answer 7

Autoimmune cholangitis is always Anti-mitochondrial antibody (AMA) negative

Question 8

Treatment for Autoimmune cholangitis.

Answer 8

Ursodiol and steroids

Question 9

Common associations with Primary Sclerosing Cholangitis (PSC).

Answer 9

1. UC (major)

2. CMV and cryptosporidium in AIDS patients

Question 10

What is Charcot’s triad.

Answer 10

Symptoms that occur with PSC.

• Elevated liver enzymes
• RUQ pain
• fever

Question 11

Diagnosis of PSC.

Answer 11

MRCP (magnetic resonance cholangiopancreatography)

-look for string of beads appearance

Question 12

Best treatment for PSC.

Answer 12

Liver Transplant

Question 13

What is Caroli’s Disease?

Answer 13

Cystic dilation of the intrahepatic bile ducts. Appears as dark spots on CT scan and results from congenital hepatic fibrosis.