PBC, PSC and AIH

Question 1

What is PBC?

Answer 1

PBC is a condition where interlobular bile ducts are damaged by chronic autoimmune granulomatous inflammation causing cholestasis. This leads to fibrosis, cirrhosis and portal hypertension.

Question 2

Which antibody is the hallmark of PBC?

Answer 2

Antimitochondrial antibodies (AMA) are the hallmark of PBC.

Question 3

What is suspected if IgG is raised?

Answer 3

Autoimmune Hepatitis AIH

Question 4

Which immunoglobulin is raised in PBC?

Answer 4

IgM

Question 5

What is suspected if ALP is raised, and ANCA is positive?

ANCA - Antineutrophil Cytoplasmic antibodies.

Answer 5

PSC - Primary Sclerosing Cholangitis

Question 6

What are the common presentation of PBC?

Answer 6

PBC is often asymptomatic. If symptomatic, fatigue and pruritus are often early symptoms that precedes jaundice.
May also present with hepatosplenomegaly, skin pigmentation, Xanthelasma and Xanthomata, Dark urine + pale stools, Dry eyes and Dry mouth

Question 7

Which ducts are affected in PBC? How about in PSC?

Answer 7

PBC: Intra-hepatic bile ducts
PSC: Both intra and extra hepatic bile ducts

Question 8

What is a histological feature (early feature) or PBC?

Answer 8

Florid duct lesions.

interlobular ducts are destroyed by granulomas, resulting in duct obliteration.

Question 9

What is IgG4 disease?

Answer 9

It is a condition which presents biochemically similar to PSC, with similar cholangiogram. However, extra-hepatic ducts are often affected only.

Question 10

Which liver condition can present with bloody diarrhoea?

Answer 10

PSC.
PSC is highly associated with Inflammatory Bowel Disease, especially Ulcerative Colitis. UC commonly presents before PSC in patients.

Question 11

What are the complications of PBC?

Answer 11

Those complications of cirrhosis:

• Hypoalbuminaemia (Leuconychia, peripheral oedema)
• Reduced clotting factors, esp fat-soluble factors II, V, VII, X –> Extrinsic pathway mainly affected, leading to increased PT.
• Hepatic encephalopathy: Asterixis and confusion/coma
• Portosystemic shunts, such as oesophageal varices and caput medusae
• Sepsis: Pneumonia, septicaemia
• Spontaneous bacterial peritonitis
• Hypoglycaemia
• Portal hypertension: Ascites, Splenomegaly
• Increased risk of HCC (Check AFP twice yearly)

Osteoporosis is also a common complication of PBC.
Malabsorption of fat soluble Vit A, D, E, K due to cholestasis and decreased bilirubin in the gut –> Osteomalacia and coagulopathy

Question 12

What are the treatment available for PBC?

Answer 12

For pruritus, give Colestyramine PO. Naltrexone or Rifampicin can be given.
For diarrhoea, give Codeine Phosphate
Osteoporosis prevention
Fat-soluble vitamin prophylaxis
Ursodeoxycholic acid

Question 13

What is the SE of Rifampicin?

Answer 13

Thrombocytopenia, Nausea and vomiting

Question 14

AIH presents in which group of patients?

Answer 14

Bimodal - predominantly affects women ages 10-30 or >40yo

Question 15

What are the auto-antibodies detected in Type I AIH?

Answer 15

Anti-nuclear antibodies (ANA) and Anti-smooth muscle antibodies (ASMA)

Question 16

Which immunoglobulin is raised in AIH?

Answer 16

IgG

Question 17

What are the presenting S/S of AIH?

Answer 17

In 40% of patients, they can present with acute hepatitis and signs of autoimmune disease, example fever, malaise, polyarthritis, pleurisy, pulmonary infiltration or glomerulonephritis.

The rest may be asymptomatic and diagnosed incidentally with signs of chronic liver disease. May have gradual jaundice.

Amenorrhoea is the most common presentation.

Question 18

What is the most common presentation in females with AIH?

Answer 18

Ameorrhoea

Question 19

When will the disease tends to attenuate in females?

Answer 19

Disease tends to attenuate in pregnancy.

Question 20

What is a characteristic finding on MRCP or ERCP in PSC?

Answer 20

Beading: multiple segmental strictures with intervening segments that are of normal calibre or slightly dilated.

“Onion skin” fibrosis: layers of periductal concentric fibrosis

Question 21

Which cancers are screened for in those with PSC?

Answer 21

Cholangiocarcinoma, Hepatocellular carcinoma and Colorectal carcinoma.