Lipoprotein Glomerulopathy

Abstract

Lipoprotein glomerulopathy is a disease characterized by intraglomerular lipoprotein thrombi and abnormal lipid metabolism similar to type III hyperlipoproteinemia. Recently, glomerular lesions compatible with lipoprotein glomerulopathy were found in a 38-year-old man. In this case, plasma apolipoprotein (apo) E level was elevated, and the apo E isoform was heterozygous E2/3, similar to most of the reported cases of lipoprotein glomerulopathy. However, the plasma levels of triglyceride and total cholesterol were within their respective normal ranges. Review in 11 cases revealed that lipoprotein glomerulopathy was not always associated with hyperlipidemia, and that apo E abnormality was always recognized. These findings suggest that lipoprotein thrombi associated with lipoprotein glomerulopathy are not induced via systemic hyperlipidemia but may be formed by intraglomerular accumulation of lipoprotein composed of abnormal apo E.

Association of Apolipoprotein E Polymorphism with Lipoprotein Glomerulopathy

“…Renal biopsy of both patients revealed the characteristic features of lipoprotein glomerulopathy as previously reported [1, 2, 3, 4, 5, 6, 7, 8, 9]. The most impressive feature was the diffuse global aneurysmal dilatation of the glomerular capillaries.…”

“…The detection of Apo B and E in capillary luminal deposits implied the possible attribution of an altered solubility of very low density lipoproteins (VLDLs) and/or VLDL remnants in the local environment causing the formation of ‘lipoprotein plugs’. There is clearly a racial predominance in oriental people, as 13 Japanese and 1 Chinese [1, 2, 3, 4, 5, 6, 7, 9] but only 2 Caucasian cases have been reported [8, 10]. In this report, we describe 2 cases of lipoprotein glomerulopathy with a new Apo E genotype (E3/E4).…”

“…Since the glomerular disease with deposition of lipoprotein ‘thrombi’ in glomeruli was first described in a review of renal lipidoses by Faraggiana et al [1] in 1987, 16 cases of this unique glomerular disease have been reported [2, 3, 4, 5, 6, 7, 8, 9, 10]. The disease is characterized by aneurysmal dilatation of glomerular capillaries with intraluminal deposition of insoluble material containing lipoproteins and lipid.…”

Lipoprotein Glomerulopathy: Renal Lipidosis Induced by Novel Apolipoprotein E Variants

“…In most cases, cholesterol in the very-low-density lipoprotein (VLDL) and in the intermediate-density lipoprotein fractions increases [28], and the lipoprotein pattern indicates type III hyperlipoproteinemia as defined by Hazzard et al [29]or Fredrickson et al [30](table 1). In addition, a plasma apo E level of more than twice the normal value has been reported in all of the investigated cases [7], although hyperlipidemia is not always recognized [18, 31]. …”

“…Kidneys were obtained from the patients’ relatives in 2 Japanese cases [7, 31]and from a cadaver in 1 French case [11]. However, all three attempts failed because of the recurrence of LPG.…”