6. Intrahepatic biliary tract diseases

Question 1

Types of intrahepatic biliary tract disease

Answer 1

1. Primary biliary cirrhosis
2. Secondary biliary cirrhosis
3. Primary sclerosing cholangitis

Question 2

Definition of primary biliary cirrhosis

Answer 2

Autoimmune disease mainly affecting the intrahepatic bile ducts; characterized by a non-suppurative inflammatory destruction of medium-sized intrahepatic bile ducts

Question 3

Morphology of primary biliary cirrhosis

Answer 3

1. Periportal fibrosis
2. Mononuclear infiltrate
3. Lymphoid follicle
4. Non-caseating granuloma
5. Non-suppurative destructive cholangitis

Question 4

Pathological Effects & Complications of primary biliary cirrhosis

Answer 4

1. Progressive inflammation, scarring & eventual cirrhosis
2. Liver failure
3. Portal hypertension & variceal bleeding
4. Increased risk of hepatocellular carcinoma

Question 5

Definition of secondary biliary cirrhosis

Answer 5

Disorder of intrahepatic bile ducts due to uncorrected extrahepatic bile duct obstruction

Question 6

Causes of secondary biliary cirrhosis

Answer 6

1. In children:
   - Biliary atresia
   - Cystic fibrosis
   - Choledochal cysts
2. In adults:
   - Gallstones
   - Carcinoma bile duct, tumours of head of pancreas or ampulla of Vater

Question 7

Morphology of secondary biliary cirrhosis

Answer 7

1. Periportal fibrosis
2. Prominent cholestasis (bile lakes)
3. Bile duct proliferation
4. Neutrophilic infiltrate
5. Intraduct neutrophils

Question 8

Pathogenesis of secondary biliary cirrhosis

Answer 8

1. Obstruction of extrahepatic bile ducts result in secondary inflammation of intrahepatic bile ducts
2. Periportal fibrosis occurs over time which eventually
   leads to hepatic scarring & nodule formation, generating secondary biliary cirrhosis
3. Subtotal obstruction of extrahepatic bile ducts may allow for ascending cholangitis, aggravating inflammation of intrahepatic bile ducts

Question 9

Definition of primary sclerosing cholangitis

Answer 9

Inflammation & obliterative fibrosis of intrahepatic & extrahepatic bile ducts, with dilation of preserved segments; produces a characteristic ‘beading’ of contrast medium in radiographs of biliary tree

Question 10

Associations with primary sclerosing cholangitis

Answer 10

Inflammatory bowel disease (Crohn’s, ulcerative colitis)

Question 11

Morphology of primary sclerosing cholangitis

Answer 11

1. Lymphocytic infiltrate
2. Progressive atrophy of bile duct epithelium
3. Obliteration of bile duct lumen
4. Concentric periductal fibrosis (onion-skin appearance) following obliteration of lumen

Question 12

Pathological Effects & Complications of primary sclerosing cholangitis

Answer 12

1. Biliary cirrhosis
2. Increased risk of cholangiocarcinoma
3. Symptoms of chronic liver disease (weight loss, ascites, encephalopathy, variceal bleeding)