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Question:

A 52-year-old woman comes to the office due to intense itching and fatigue. She is unable to specify when her symptoms started as they developed gradually. Past medical history is significant for hypothyroidism and carpal tunnel syndrome. Current medications include levothyroxine. The patient lives with her husband and 3 children. She does not smoke and drinks wine on social occasions. Vital signs are normal. Cardiopulmonary examination shows no abnormalities. The abdomen is soft with normal bowel sounds. Hepatomegaly is present. There is no scleral icterus or jaundice, but bilateral xanthelasma and skin excoriations are evident. Laboratory results are as follows:

A right upper quadrant ultrasound shows a normal common bile duct. Which of the following is the most appropriate next step in management of this patient?

Answers:

Correct answer:

A. Check anti-mitochondrial antibodies

B. Anti-smooth muscle antibodies

Your reply:

C. Discontinue levothyroxine

D. Abdominal MRI

E. Prescribe oral glucocorticoids

Explanation:

Educational objective: Primary biliary cholangitis (previously termed primary biliary cirrhosis) is a chronic liver disease characterized by intrahepatic cholestasis due to autoimmune destruction of small bile ducts. It presents in middle-aged women with fatigue, pruritus, hepatomegaly, and elevated alkaline phosphatase. The diagnosis is confirmed with serum anti-mitochondrial antibody titers.

Choice B and E. Autoimmune hepatitis is associated with elevated titers of antinuclear antibodies and anti-smooth muscle antibodies. It is characterized by fluctuating hepatocellular injury (ie, elevated transaminases) rather than cholestasis. First-line treatment includes oral glucocorticoids.

Choice C. A number of medications can cause intrahepatic cholestasis, including certain antibiotics (eg, macrolides), anabolic steroids, and oral contraceptives. Levothyroxine is not associated with cholestasis.

Choice D. Abdominal MRI is primarily used to evaluate abdominal masses or clarify nonspecific abnormalities on other imaging tests. It is not needed in the evaluation of PBC.

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