PBC/PSC/AIH

Question 1

Autoimmune-mediated liver disease (positive AMA); lymphocytes destroy SMALL intrahepatic bile ducts; buildup of toxic constituents of bile lead to inflammation of portal areas and granulomas (florid duct lesions); can progress to cholestasis, fibrosis and cirrhosis

Answer 1

Primary Biliary Cholangitis (PBC)

Question 2

Primary Biliary Cholangitis is much more common in (men/women)

Answer 2

women

Question 3

Histologic features of Primary Biliary Cholangitis (PBC)

Answer 3

Florid duct lesions (lymphocyte infilate + granulomas)

Question 4

90-95% of patients with Primary Biliary Cholangitis (PBC) have what antibodies?

Answer 4

Anti-Mitochondrial Antibodies (AMA)

Question 5

Clinical presentation of PBC

Answer 5

Fatigue
Pruritus (due to bile accumulation in skin)
Jaundice
Xanthelasma

Question 6

Xanthelasma

Answer 6

yellow discoloration around eyes (cholesterol deposits caused by PBC)

Question 7

How to diagnose Primary Biliary Cholangitis

Answer 7

• Elevated Alkaline Phosphatase
• AMA +
• Biopsy (florid duct lesion)

Question 8

PBC mainly affects (small/large) bile ducts while PSC mainly affects (small/large) bile ducts

Answer 8

PBC: small
PSC: med/large

Question 9

chronic INFLAMMATORY, cholestatic disease affecting LARGE bile ducts with progressive cholestasis followed by biliary fibrosis and cirrhosis; concentric periductal fibrosis (ONION-SKINNING), with narrowing or TOTAL OBLITERATION of the bile duct lumen, correlating with the beaded appearance; cholestasis and cirrhosis will follow

Answer 9

Primary Sclerosing Cholangitis (PSC)

Question 10

Primary Sclerosing Cholangitis is more common in (men/women)

Answer 10

Men

Question 11

Primary Sclerosing Cholangitis is often seen in those who also have what intestinal disease

Answer 11

IBD (specifically UC)

Question 12

Histologic appearance of Primary Sclerosing Cholangitis

Answer 12

• Onion-skinning (concentric periductal fibrosis)

- Dysplasia (can become cholangiocarcinoma)

Question 13

Clinical presentation of PSC

Answer 13

• Abdominal pain (due to stricture from fibrosis)
• Fatigue
• Pruritus (due to bile accumulation in skin)
• Jaundice

Question 14

Bile duct dysplasia can be a possibility in (PBC/PSC)

Answer 14

PSC

Question 15

What will cholangiogram show in PSC?

Answer 15

string of beads (due to stricture)

Question 16

(PBC/PSC) can be seen to have completely obliterated/fibrosis-filled bile ducts

Answer 16

PSC (Primary Sclerosing Cholangitis)

Question 17

How to diagnose Primary Sclerosing Cholangitis

Answer 17

• Elevated Alkaline Phos
• p-ANCA +
• Cholangiogram (string of beads)
• Biopsy (onion-skinning and dysplasia)

Question 18

Treatment for PSC

Answer 18

Medical treatment is not effective. In fact, it can be more toxic!

Question 19

autoimmune attack on hepatocytes; characterized by numerous plasma cells throughout tissue; acidophils are numerous (apoptotic cells); can progress to cirrhosis if untreated with immunosuppressive therapy

Answer 19

Autoimmune Hepatitis

Question 20

AI Hepatitis usually results in (hepatocyte/bile duct) injury while PBC/PSC usually results in (hepatocyte/bile duct) injury

Answer 20

• AI Hepatitis: hepatocyte injury

- PBC/PSC: Cholestatic (bile duct) injury

Question 21

Clinical presentation of PSC

Answer 21

• Fatigue
• Pruritus (due to bile accumulation in skin)
• Jaundice
• Arthralgia (joint pain)

Question 22

Autoimmune Hepatitis is usually more common in (men/women)

Answer 22

Women

Question 23

How do you diagnose Autoimmune Hepatitis

Answer 23

• Elevated AST/ALT
• ASMA+
• Elevated ANA

Question 24

Unique Marker for PBC, PSC, and AI

Answer 24

• PBC: AMA
• PSC: p-ANCA
• AI: ASMA

Question 25

Treatment for Autoimmune Hepatitis

Answer 25

Immunosuppression (steroids)