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Diagnosis: Lipoprotein Glomerulopathy

Since it was first described in 1989, lipoprotein glomerulopathy (LPG) has been characterized as a rare glomerular disorder leading to nephrotic syndrome and/or kidney failure. LPG is characterized clinically by proteinuria and elevated concentrations of triglyceride-rich lipoproteins and their remnants, and histologically characterized by lamellated lipoprotein "thrombi" in glomerular capillary lumina lacking foam cells. The familial occurrence of LPG has been frequently recognized. LPG is primarily associated with heterozygous APOE mutations in the low-density lipoprotein–receptor binding site or around it. As a “Mendelian disease” caused by a “single gene” with dominant inherited disease of incomplete penetrance, it also provides a disease model to explore pathogenic roles of APOE in some common diseases, such as Alzheimer's disease, type III hyperlipoproteinemia (HLP), and coronary artery disease. Trending evidence suggests that APOE gene mutations play an important role by potentially increasing the affinity of lipoproteins for the glomerular capillary wall or by enhancing the tendency of mutant apolipoproteins to form aggregates when concentrated. If left untreated, the disease usually progresses to end-stage kidney disease. Lipid-lowering medications, especially fibrates, were found to improve both clinical manifestations and histological alterations. In recent years, there has been a rapid increase in the number of LPG cases reported, and some progress in research into the mechanism and animal models of LPG (Li MS, et al. An Updated Review and Meta Analysis of Lipoprotein Glomerulopathy. Front Med (Lausanne). 2022;9:905007. [PubMed link]).

See the chapter: Hereditary Diseases of our Tutorial.

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