A middle-aged woman presents to her physician with fatigue, pruritus, and steatorrhea. Fuher studies demonstrate sclerosing cholangitis and the presence of antimitochondrial antibodies. This patient, if left untreated, is also at increased risk of developing which of the following?
Correct Answer: Markedly increased serum cholesterol
Description: The patient's disease is primary biliary cirrhosis, which is an autoimmune disease characterized by sclerosing cholangitis and cholangitis. The most helpful clue in the question stem is the antimitochondrial antibody, which is present in over 90% of the patients. The course is usually slowly progressive over 5-25 years. Patients are sometimes diagnosed when routine blood tests demonstrate an elevated alkaline phosphatase. In addition to the features listed in the question, patients with advanced disease tend to develop profound hypercholesterolemia and xanthomas. Ref: Wyatt C., Butterwoh IV J.F., Moos P.J., Mackey D.C., Brown T.G. (2008). Chapter 15. Pathology of the Liver, Gallbladder, and Pancreas. In C. Wyatt, J.F. Butterwoh IV, P.J. Moos, D.C. Mackey, T.G. Brown (Eds), Pathology: The Big Picture.
Category:
Pathology
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