Chapter 18 –Intrahepatic Biliary Tract Disease

Question 1

In this section we discuss three disorders of intrahepatic bile ducts:

• secondary biliary cirrhosis ,
• cholangitis (summarized in Table 18-8 ).
• primary biliary cirrhosis ,
• and primary sclerosing

Question 2

What is Secondary biliary cirrhosis?

Answer 2

is a condition resulting most often from uncorrected obstruction of
the extrahepatic biliary tree.

Question 3

What is Primary biliary cirrhosis?

Answer 3

is a destructive disorder of the intrahepatic biliary tree.

Question 4

What is Primary selerosing cholangitis?

Answer 4

involves both the extrahepatic and intrahepatic biliary
tree.

It should also be noted that intrahepatic bile ducts are frequently damaged as part of more general liver diseases as in drug toxicity, viral hepatitis, liver transplantation, and graft-versushost
disease after bone marrow transplantation

Question 5

TABLE 18-8 – Distinguishing Features of the Major Intrahepatic Bile Duct Disorders

Etiology

Answer 5

• Secondary Biliary Cirrhosis
  
  • Extrahepatic bile duct
    obstruction:
  • biliary atresia, gallstones, stricture,
    carcinoma of pancreatic head
• Primary Billiary Cirrhosis
  
  • Possibly autoimmune
• Primary Sclerosing Cholangitis
  
  • Unknown, possibly
    autoimmune;
  • 50% to 70% associated with inflammatory bowel disease

Question 6

TABLE 18-8 – Distinguishing Features of the Major Intrahepatic Bile Duct Disorders

Sex
predilection

Answer 6

• Secondary Biliary Cirrhosis
  
  • None
• Primary Billiary Cirrhosis
  
  • Female to male, 6 : 1
• Primary Sclerosing Cholangitis
  
  • Female to male, 1 : 2

Question 7

TABLE 18-8 – Distinguishing Features of the Major Intrahepatic Bile Duct Disorders

Symptoms
and signs

Answer 7

• Secondary Biliary Cirrhosis
  
  • Pruritus, jaundice,
    malaise, dark urine, light stools, hepatosplenomegaly
• Primary Billiary Cirrhosis
  
  • Same as secondary biliary
    cirrhosis;
  • insidious onset
• Primary Sclerosing Cholangitis
  
  • Same as secondary biliary cirrhosis;
  • insidious
    onset

Question 8

TABLE 18-8 – Distinguishing Features of the Major Intrahepatic Bile Duct Disorders

Laboratory
findings

Answer 8

• Secondary Biliary Cirrhosis
  
  • Conjugated hyperbilirubinemia,
  • increased serum alkaline
    phosphatase, bile acids, cholesterol
• Primary Billiary Cirrhosis
  
  • ​Same as secondary biliary
    cirrhosis,
  • plus elevated serum IgM autoantibodies (especially M2 form of anti-mitochondrial
    antibody)
• Primary Sclerosing Cholangitis
  
  • ​Same as secondary biliary cirrhosis,
  • plus elevated serum IgM,
  • hypergammaglobulinemia

Question 9

TABLE 18-8 – Distinguishing Features of the Major Intrahepatic Bile Duct Disorders

Important pathologic findings before cirrhosis
develops

Answer 9

• Secondary Biliary Cirrhosis
  
  • ​Prominent bile stasis in bile ducts, bile ductular
    proliferation with surrounding neutrophils,
    portal tract edema
• Primary Billiary Cirrhosis
  
  • ​Dense lymphocytic infiltrate in portal tracts with granulomatous destruction of bile ducts
• Primary Sclerosing Cholangitis
  
  • ​Periductal portal tracts fibrosis,
  • segmental stenosis of extrahepatic and intrahepatic bile ducts