Extreme hypercholesterolemia in cholestatic sarcoidosis due to lipoprotein X: Exploring the cholesterol gap

Abstract

Lipoprotein-X (LpX) is a rare lipid particle that can affect lipid analysis and interpretation. Case reports of LpX syndrome have been described scarcely. LpX has been linked to diseases associated with cholangiopathy and thus, in such instances, hyperlipidemia can be falsely attributed to an elevated LDL-cholesterol component or to an unknown lipid moiety. The management of LpX syndrome is completely different than that of classic hypercholesterolemia. This article describes a case of LpX syndrome and provides a review of similar cases in the literature. This review also discusses the technical measurement of LpX, management expectations and sequelae of the disease.