Question 147

Question:

A 67-year old woman presents with complaints of pruritis which has developed over several months. Physical exam is notable only for yellow-tinged sclera. Laboratory values reveal an increased direct bilirubin. What is her diagnosis?

Correct Answer: B.  Primary Biliary Cirrhosis

Primary biliary cirrhosis (PBC) results in a direct hyperbilirubinemia through fibrosis of the biliary tree resulting in cholestasis. Anti-mitochondrial antibodies are usually present. Pruritus is usually the first symptom, jaundice develops later.
Gilbert syndrome and Crigler-Najar types 1 and 2 would result in an indirect hyperbilirubinemia due to a defect in UDP-glucuronosyltransferase which conjugates bilirubin. Of note, Crigler-Najar type 1 usually results in kernicterus in the neonatal period.
Glucose-6-phosphate dehydrogenase (G6PD) deficiency would also result in an indirect hyperbilirubinemia due to hemolysis.
This patient’s presentation is not consistent with acute cholecystitis given time course and lack of fever or abdominal pain.