Autoimmunity and the liver

Question 1

Who is most likely to be a carrier for Hep B?

Answer 1

Neonates. Immature immune system is less likely to clear the infection.

Question 2

What HLA types is autoimmune hepatitis associated with?

Answer 2

HLA-DR3 and HLA-DR4

Question 3

What autoantibodies associated with AIH?

Answer 3

1. ANA
2. SMA (smooth muscle antibodies)
3. Liver-kidney microsomal

Question 4

What unique labs would you see in AIH?

Answer 4

bilirubin high
--Elevated transaminases (500-1000)
--HYPER GAMMA globulinemia
--positive for autoantibodies
HOWEVER, often asymptomatic. Can co-exist with other autoimmune diseases

Question 5

Presentation of AIH?

Answer 5

Same as general signs of hepatitis

• -Fatigue
• -Dark urine/light stools
• -RUQ discomfort
• -Arthralgia
• -spider nevi
• -Jaundice
• -splenomegaly/hepatomegaly

Question 6

Unique findings on biopsy of autoimmune hepatitis:

Answer 6

1. PLASMA cells

2. Lobular inflammation with rosettes

Question 7

What could help in your diagnosis of AIH?

Answer 7

Use the scoring system from autoimmune hepatitis group. Definite AIH if you score over 15.

Question 8

How do you treat autoimmune hepatitis?

Answer 8

Prednisone

-Azathioprine

Question 9

What’s on your differential with a transaminase level above 1000?

Answer 9

1. Acute viral hepatitis
2. Autoimmune hepatitis
3. Ischemic hepatitis
4. Acute drug/toxin injury

Question 10

What’s on your differential with a transaminase > 10,000

Answer 10

acetaminophen toxicity

ischemic hepatitis

Question 11

What liver lab finding would make you suspect alcoholic hepatitis?

Answer 11

AST: ALT ratio of 2:1

Question 12

Typical pt with primary biliary cirrhosis?

Answer 12

Middle aged women

Question 13

Pathogenesis of primary biliary cirrhosis?

Answer 13

Destruction of interlobular bile ducts (at the microscopic level).

• -Ductopenia
• -Slowly progressive cholestasis

Question 14

Symptoms of Primary Biliary cirrhosis?

Answer 14

Hyperpigmentation
--Pruritis
--Xanthomas 
--Jaundice is a late manifestation
Often, normal PE

Question 15

What lab would you want to order in PBC?

Answer 15

AMA is sensitive and specific for PBC

Question 16

Histology of PBC

Answer 16

FLORID DUCT LESION is textbook.

–Portal triad with inflammatory cells

Question 17

How do you treat PBC?

Answer 17

Ursodeoxycholic acid

Question 18

Typical pt with primary sclerosing cholangitis?

Answer 18

Males much more common

Question 19

Pathogenesis of primary sclerosing cholangitis

Answer 19

Inflammation, fibrosis, and stricturing of medium nad large bile ducts (as opposed to PBC)

Question 20

What other disease do you need to watch out for in a pt with primary sclerosing cholangitis?

Answer 20

Ulcerative colitis. If they have UC, then at risk for colon cancer

Question 21

What are complications of primary sclerosing cholangitis?

Answer 21

1. Cholestasis
2. Biliary strictures
3. Bacterial cholangitis
4. Cholangiocarcinoma

Question 22

Histological findings in primary sclerosing cholangitis?

Answer 22

Onion skinning

Question 23

How can you tell whether elevated GGT/AP is due to biliary obstruction VERSUS hepatic cholestasis?

Answer 23

Need to image the liver/biliary tree with ultrasound
PSC==Biliary obstruction (larger bile ducts)
PBC:==hepatic cholestasis