title: Management of lipoprotein X and its complications in a patient with primary sclerosing cholangitis.
abstract: Lipoprotein X (LpX) is an abnormal lipoprotein found in conditions such as lecithin:cholesterol acyltransferase deficiency and cholestatic states (e.g., primary biliary cirrhosis and primary sclerosing cholangitis). Management of severe hypercholesterolemia due to LpX with drugs and physical removal methods is not well established in the literature. A case is discussed of a 51-year-old woman who presented with multiple electrolyte abnormalities, xanthomas and neuropathy found to be secondary to LpX in the setting of primary sclerosing cholangitis. This case highlights that oral medications, including statins, may be insufficient to normalize lipid levels or improve clinical symptoms of LpX and presents therapeutic plasma exchange as a safe and effective therapeutic option to treat the morbid sequela of LpX hyperlipidemia.
authors.affiliation: Department of Medicine, University of Chicago, Chicago, IL, 60637,USA.
authors.forename: Eric J
authors.initials: EJ
authors.lastname: Brandt
authors.affiliation: Pritzker School of Medicine, University of Chicago, Chicago, IL 60637, USA.
authors.forename: Shane M
authors.initials: SM
authors.lastname: Regnier
authors.affiliation: Pritzker School of Medicine, University of Chicago, Chicago, IL 60637, USA ; Department of Pathology, University of Chicago, Chicago, IL, 60637,USA.
authors.forename: Edward Ky
authors.initials: EK
authors.lastname: Leung
authors.affiliation: Department of Medicine, University of Chicago, Chicago, IL, 60637,USA ; Section of Endocrinology, Diabetes & Metabolism, University of Chicago, Chicago, IL, 60637,USA.
authors.forename: Sharon H
authors.initials: SH
authors.lastname: Chou
authors.affiliation: Pritzker School of Medicine, University of Chicago, Chicago, IL 60637, USA ; Department of Pathology, University of Chicago, Chicago, IL, 60637,USA.
authors.forename: Beverly W
authors.initials: BW
authors.lastname: Baron
authors.affiliation: Pritzker School of Medicine, University of Chicago, Chicago, IL 60637, USA ; Section of Gastroenterology, University of Chicago, Chicago, IL, 60637,USA.
authors.forename: Helen S
authors.initials: HS
authors.lastname: Te
authors.affiliation: Pritzker School of Medicine, University of Chicago, Chicago, IL 60637, USA ; Section of Cardiology, University of Chicago, Chicago, IL, 60637,USA.
authors.forename: Michael H
authors.initials: MH
authors.lastname: Davidson
authors.affiliation: Department of Medicine, University of Chicago, Chicago, IL, 60637,USA ; Pritzker School of Medicine, University of Chicago, Chicago, IL 60637, USA ; Section of Endocrinology, Diabetes & Metabolism, University of Chicago, Chicago, IL, 60637,USA.
authors.forename: Robert M
authors.initials: RM
authors.lastname: Sargis
keywords: cholestasis; hypercholesterolemia; lipoprotein X; neuropathy; plasma exchange; primary sclerosing cholangitis; statin; xanthoma