Lipoprotein glomerulopathy: what is known? Literature review

The review is devoted to the important problem of contemporary nephrology — lipoprotein glomerulopathy (LPG), a rare glomerular disorder leading to nephrotic syndrome and/or chronic kidney failure (CKF). The article highlights the primary genetic defect in LPG associated with heterozygous mutations of the apolipoprotein E (ApoE) gene in the low‑density lipoprotein (LDL) binding site with the corresponding receptor, which increases the affinity of mutant ApoE‑containing lipoproteins to the glomerular capillary wall. The article presents data from population‑based studies on the prevalence of LPG and current understanding of its pathogenesis, in particular, the main pathogenetic link — damage to the structure and function of ApoE due to heterozygous mutations of the ApoE gene. The participation of ApoE‑containing lipoproteins, which include ApoE isoforms, in the formation of glomerular deposits, lipoprotein thrombi in the lumen of glomerular capillaries, as well as the role of macrophages in the pathogenesis of renal lipidosis is considered. Histological picture typical for LPG, intraglomerular and extrarenal factors of its development, and features of serum lipid spectrum accompanying the clinical course of LPG are described. Diagnostic criteria of LPG, clinical manifestations of this glomerular disorder, prognosis in LPG, its complications and some co-morbid states associated with LPG are given. Special attention is paid for the treatment of LPG, without which the disease is progressing to a terminal CKF. Some possibilities of contemporary lipid lowering agents (fibrates, nicotinic acid derivates, statins, monoclonal antibodies to apolipoprotein CIII) in correction of lipid disorders associated with LPG are discussed. Some perspective agents that could be applied in patients with LPG (inhibitors of natrium-dependent glucose co-transporter 2, antioxidants, anticonvulsant topiramate) after special trials are enumerated. Some attention is paid to other treatment measures in LPG, such as LDL-apheresis with heparin‑induced extracorporal precipitation, staphylococcal protein-A immunoadsorption therapy, and renal transplantation. Necessity is emphasized of further experimental and clinical studies that will allow to enlarge updated ideas about the etiology and pathogenesis of LPG, improve its diagnostics and treatment efficacy.