GE: Portuguese Journal of Gastroenterology (Oct 2022)

Severe Hypercholesterolemia Mediated by Lipoprotein X in an Immunosuppressed Patient: A Case Report

  • Cristiana Sequeira,
  • Mariana Coelho,
  • Inês Costa Santos,
  • Sara Ramos Lopes,
  • João Mangualde,
  • Ana Paula Oliveira

DOI
https://doi.org/10.1159/000526854

Abstract

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Cholestatic liver diseases may be associated with increased plasmatic cholesterol due to an abnormal lipoprotein – lipoprotein X (LpX). Correcting the underlying cause of cholestasis is the critical treatment of LpX-associated hypercholesterolemia without any proven benefit from conventional lipid-lowering agents. In some situations, plasma exchange may apply to prevent associated complications, such as hyperviscosity syndrome. The authors present the case of a 44-year-old man with orbital inflammatory pseudotumor on prednisolone, admitted due to hepatocellular and cholestatic lesion and severe hypercholesterolemia. Laboratory investigation established that hepatitis E virus was responsible for liver injury and showed that LpX mediated the severe hypercholesterolemia. Reduction of the immunosuppressive load contributed to virus clearance. The consequent resolution of cholestasis and cholesterol removal by plasmapheresis allowed lipid profile normalization. The authors report the first case of LpX-associated hypercholesterolemia in a patient with hepatitis E-induced cholestasis and revisit the role of the liver in lipid metabolism.

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