A 43-year-old man has experienced progressive fatigue, pruritus, and icterus for 4 months. A colectomy was performed 5 years ago for the treatment of ulcerative colitis. On physical examination, he now has generalized jaundice. The abdomen is not distended; on palpation, there is no abdominal pain and there are no masses. Laboratory studies show a serum alkaline phosphatase level of 285 U/L and an elevated titer of anti-neutrophil cytoplasmic antibodies. Cholangiography shows widespread intrahepatic biliary tree obliteration and a beaded appearance in the remaining ducts. Which of the following morphologic features is most likely to be present in his liver?
A 43-year-old man has experienced progressive fatigue, pruritus, and icterus for 4 months. A colectomy was performed 5 years ago for the treatment of ulcerative colitis. On physical examination, he now has generalized jaundice. The abdomen is not distended; on palpation, there is no abdominal pain and there are no masses. Laboratory studies show a serum alkaline phosphatase level of 285 U/L and an elevated titer of anti-neutrophil cytoplasmic antibodies. Cholangiography shows widespread intrahepatic biliary tree obliteration and a beaded appearance in the remaining ducts. Which of the following morphologic features is most likely to be present in his liver?
π‘ Explanation
**Core Concept**
The patient's symptoms and laboratory results suggest primary sclerosing cholangitis (PSC), a chronic liver disease characterized by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. This leads to bile duct obstruction, cholestasis, and ultimately liver cirrhosis.
**Why the Correct Answer is Right**
In PSC, the chronic inflammation and fibrosis cause the bile ducts to become obliterated and narrowed, resulting in a beaded appearance on cholangiography. The obliteration of the bile ducts leads to bile stasis, which in turn causes liver damage and cirrhosis. The elevated serum alkaline phosphatase level is a marker of cholestasis, which is consistent with PSC.
**Why Each Wrong Option is Incorrect**
**Option A:** Focal nodular hyperplasia is a benign liver lesion that can cause liver dysfunction, but it would not account for the widespread bile duct obliteration and beaded appearance seen in PSC.
**Option B:** Cholangiocarcinoma is a type of bile duct cancer that can occur in PSC patients, but it would not be the primary morphologic feature in this case.
**Option C:** Primary biliary cirrhosis is another chronic liver disease, but it primarily affects the intrahepatic bile ducts, whereas PSC affects both intrahepatic and extrahepatic bile ducts.
**Option D:** Wilson's disease is a genetic disorder that causes copper accumulation in the liver, but it would not account for the bile duct obliteration and beaded appearance seen in PSC.
**Clinical Pearl / High-Yield Fact**
Primary sclerosing cholangitis is strongly associated with inflammatory bowel disease (IBD), particularly ulcerative colitis, and patients with IBD should be screened for PSC with regular liver function tests and imaging studies.
**Correct Answer:** C.
β Correct Answer: A. Concentric "onion-skin" ductular fibrosis
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