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JAMA Cardiology Clinical Challenge
May 2017

Treatment of Severe Hypercholesterolemia in a Woman With Advanced Primary Sclerosing Cholangitis

Author Affiliations
  • 1Department of Medicine, University of Toronto, Toronto, ON, Canada
  • 2Department of Physiology, University of Toronto, Toronto, ON, Canada
  • 3Banting and Best Diabetes Centre, University of Toronto, Toronto, ON, Canada
  • 4Robarts Research Institute, Schulich School of Medicine and Dentistry, Western University, London, ON, Canada
JAMA Cardiol. 2017;2(5):575-576. doi:10.1001/jamacardio.2017.0263

A 30-year-old woman was diagnosed with primary sclerosing cholangitis (PSC) during pregnancy at age 25 years. She was normocholesterolemic prior to the diagnosis of PSC (total cholesterol [TC], 188 mg/dL [to convert to millimoles per liter, multiply by 0.0259]). Following the diagnosis of PSC, she developed moderate hypercholesterolemia (representative TC, 256 mg/dL). She first noted the appearance of periorbital xanthelasmata 2 years after PSC diagnosis (Figure 1A). Approximately 6 months prior to referral, she developed marked worsening of biliary obstruction, deterioration of liver function, and clinical jaundice (aspartate aminotransferase, 145 U/L; alanine aminotransferase, 111 U/L; alkaline phosphatase, 726 U/L; and bilirubin, 6.7mg/dL), and she noted palmar xanthomata in the creases of her palms and fingers (Figure 1B).

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