Chapter 18 –Intrahepatic Biliary Tract Disease Flashcards
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
What is Secondary biliary cirrhosis?
is a condition resulting most often from uncorrected obstruction of
the extrahepatic biliary tree.
What is Primary biliary cirrhosis?
is a destructive disorder of the intrahepatic biliary tree.
What is Primary selerosing cholangitis?
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
TABLE 18-8 – Distinguishing Features of the Major Intrahepatic Bile Duct Disorders
Etiology
-
Secondary Biliary Cirrhosis
- Extrahepatic bile duct
obstruction: - biliary atresia, gallstones, stricture,
carcinoma of pancreatic head
- Extrahepatic bile duct
-
Primary Billiary Cirrhosis
- Possibly autoimmune
-
Primary Sclerosing Cholangitis
- Unknown, possibly
autoimmune; - 50% to 70% associated with inflammatory bowel disease
- Unknown, possibly
TABLE 18-8 – Distinguishing Features of the Major Intrahepatic Bile Duct Disorders
Sex
predilection
-
Secondary Biliary Cirrhosis
- None
-
Primary Billiary Cirrhosis
- Female to male, 6 : 1
-
Primary Sclerosing Cholangitis
- Female to male, 1 : 2
TABLE 18-8 – Distinguishing Features of the Major Intrahepatic Bile Duct Disorders
Symptoms
and signs
-
Secondary Biliary Cirrhosis
- Pruritus, jaundice,
malaise, dark urine, light stools, hepatosplenomegaly
- Pruritus, jaundice,
-
Primary Billiary Cirrhosis
- Same as secondary biliary
cirrhosis; - insidious onset
- Same as secondary biliary
-
Primary Sclerosing Cholangitis
- Same as secondary biliary cirrhosis;
- insidious
onset
TABLE 18-8 – Distinguishing Features of the Major Intrahepatic Bile Duct Disorders
Laboratory
findings
-
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)
- Same as secondary biliary
-
Primary Sclerosing Cholangitis
- Same as secondary biliary cirrhosis,
- plus elevated serum IgM,
- hypergammaglobulinemia
TABLE 18-8 – Distinguishing Features of the Major Intrahepatic Bile Duct Disorders
Important pathologic findings before cirrhosis
develops
-
Secondary Biliary Cirrhosis
- Prominent bile stasis in bile ducts, bile ductular
proliferation with surrounding neutrophils,
portal tract edema
- Prominent bile stasis in bile ducts, bile ductular
-
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