Lipoprotein Glomerulopathy: Glomerular Lipoprotein Thrombi in a Patient With Hyperlipoproteinemia
Abstract
An unusual nephropathy presumably induced by abnormal lipid metabolism is described in a 57-year-old woman who presented with proteinuria and edema. Histology at renal biopsy was characterized by marked dilatation of capillary lumina. Sudan staining and electron microscopy demonstrated lipid droplets occupying the capillary lumina. The patient had no particular clinical symptoms of lipidosis, but hyperlipoproteinemia similar to type III was suggested by lipid profiles. The nephropathy is thought to be an inherited disorder because proteinuria was detected in her sisters and similar renal histology and lipid profile were observed in one of her sisters. This is believed to be the first detailed report of glomerular lipoprotein thrombi.
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Lipoprotein glomerulopathy (LPG) is associated with various heterozygous ApoE variants, such as ApoE Sendai and ApoE Kyoto.
“Lipoprotein glomerulopathy (LPG) was initially reported in Japan [2] and later known to be associated with various heterozygous ApoE variants, e.g., ApoE Sendai [3] and ApoE Kyoto [4].”
From Membranous Nephropathy-Like Apolipoprotein E Deposition Disease with Apolipoprotein E Toyonaka (Ser197Cys) and a Homozygous Apolipoprotein E2/2 (2018) · cites this paper as [2]
Diagnosis of LPG relies primarily on renal pathology, with typical lipoprotein thrombosis within dilated glomerular capillary loops.
“Diagnosis of LPG relies primarily on renal pathology, with typical lipoprotein thrombosis within dilated glomerular capillary loops.”
From A case report of youth-onset lipoprotein glomerulopathy with APOE Chicago mutation (2024) · cites this paper as [1]
Lipoprotein glomerulopathy (LPG) is an inherited glomerular disease caused by mutations in the APOE gene.
“Lipoprotein glomerulopathy (LPG) is an inherited glomerular disease caused by mutations in the APOE gene, usually with clinical manifestations of dysregulated lipid metabolism, proteinuria, and renal insufficiency.”
From A case report of youth-onset lipoprotein glomerulopathy with APOE Chicago mutation (2024) · cites this paper as [1]
The first 2 cases of LPG were reported by Saito et al. in the same family in 1989.
“The first 2 cases of LPG were reported by Japanese scholar Saito et al. in the same family in 1989 [1].”
From A case report of youth-onset lipoprotein glomerulopathy with APOE Chicago mutation (2024) · cites this paper as [1]
Massive lipoprotein thrombi in the lumens of dilated glomerular capillaries are the typical pathological manifestation of LPG.
“Massive lipoprotein thrombi in the lumens of dilated glomerular capillaries are the typical pathological manifestation of LPG3).”
From The Novel Apolipoprotein E Mutation ApoE Chengdu (c.518T＞C, p.L173P) in a Chinese Patient with Lipoprotein Glomerulopathy (2018) · cites this paper as [3]
In contrast to apoE2 homozygote glomerulopathy, LPG is characterized by dilation of glomerular capillaries with lipoprotein thrombi showing lamellar formation, directly inducing glomerular damage without inducing foamy changes.
“In contrast to apoE2 homozygote glomerulopathy, LPG is characterized by dilation of glomerular capillaries with lipoprotein thrombi showing lamellar formation, directly inducing glomerular damage without inducing foamy changes68).”
From Impact of Apolipoprotein E Variants: A Review of Naturally Occurring Variants and Clinical Features (2025) · cites this paper as [68]
LPG is a rare renal disease first reported in Japan in 1989.
“LPG is a rare renal disease first reported in Japan in 1989 68).”
From Impact of Apolipoprotein E Variants: A Review of Naturally Occurring Variants and Clinical Features (2025) · cites this paper as [68]
LPG was initially identified as a glomerular disease associated with type III HLP.
“LPG was initially identified as a glomerular disease associated with type III HLP68).”
From Impact of Apolipoprotein E Variants: A Review of Naturally Occurring Variants and Clinical Features (2025) · cites this paper as [68]
Histological features are the most important evidence for the diagnosis of lipoprotein glomerulopathy.
“Histological features are the most important evidence for the diagnosis of LPG [2].”
From A novel apolipoprotein E mutation, ApoE Ganzhou (Arg43Cys), in a Chinese son and his father with lipoprotein glomerulopathy: two case reports (2022) · cites this paper as [2]
Immunofluorescence microscopy in lipoprotein glomerulopathy shows positive staining of ApoE and ApoB in lumen thrombotic substances.
“Immunofluorescence microscopy shows positive staining of ApoE and ApoB in lumen thrombotic substances.”
From A novel apolipoprotein E mutation, ApoE Ganzhou (Arg43Cys), in a Chinese son and his father with lipoprotein glomerulopathy: two case reports (2022) · cites this paper as [2]
Nearly half of reported ApoE mutations in lipoprotein glomerulopathy occurred in the LDL-receptor binding site, and these mutations might result in reduced binding capacity of ApoE protein to the LDL receptor.
“Nearly half of mutations occurred in the LDL-receptor binding site (LRBS), such as ApoE Sendai (Arg145Pro) [2], ApoE Chicago (Arg147Cys) [16], ApoE Guangzhou (Arg150Pro)[17], ApoE Modena (Arg 150Cys) [11], ApoE Okayama (Arg150Gly) [18], ApoE Tokyo (Del 141-143) [19], and ApoE Maebasi (Del 142-144). These mutations might result in reduced binding capacity of ApoE protein to LDL receptor.”
From A novel apolipoprotein E mutation, ApoE Ganzhou (Arg43Cys), in a Chinese son and his father with lipoprotein glomerulopathy: two case reports (2022) · cites this paper as [2]
Under light microscopy, the capillary lumen in lipoprotein glomerulopathy is typically highly dilated and filled with lightly stained reticular material.
“Under light microscopy, the capillary lumen is typically highly dilated and filled with lightly stained reticular material.”
From A novel apolipoprotein E mutation, ApoE Ganzhou (Arg43Cys), in a Chinese son and his father with lipoprotein glomerulopathy: two case reports (2022) · cites this paper as [2]
Lipoprotein glomerulopathy (LPG) is a rare inherited disease characterized by lipoprotein thrombi in dilated glomerular capillaries and type III-like hyperlipoproteinemia with heterozygous APOE mutation.
“Lipoprotein glomerulopathy (LPG) is a rare inherited disease characterized by histopathological features of lipoprotein thrombi in dilated glomerular capillaries and type III like hyperlipoproteinemia with heterozygous mutation of the apolipoprotein (apo) E gene.”
From A case of lipoprotein glomerulopathy with a rare apolipoprotein E isoform combined with neurofibromatosis type I (2018) · cites this paper as [1]
LPG was first described in Japan in 1989.
“Lipoprotein glomerulopathy (LPG) is a condition first described in Japan in 1989.1”
From Lipoprotein Glomerulopathy, First Case Report from Canada (2022) · cites this paper as [1]
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Self-citation
- Authors
- Saito, T., Sato, H., Kudo, K.-I., Oikawa, S.-I., Shibata, T., Hara, Y., Yoshinaga, K., & Sakaguchi, H.
- Affiliations
- Tohoku University (JP)
- Corresponding author
- Saito, Takao
- Venue
- American Journal of Kidney Diseases
- Publisher
- Elsevier BV
- Published
- 1989-02
- Volume
- 13
- Issue
- 2
- Pages
- 148-153
- ISSN
- 0272-6386 (ISSN-L)
- OpenAlex
- W1964757582
- Semantic Scholar
- da403efad9eaea0bf6dbcbfdb6bf2659875fab39
- Cited by
- 148
- References
- 30
- Citations by year
- 2026: 4 · 2025: 5 · 2024: 4 · 2023: 3 · 2022: 5 · 2021: 4 · 2020: 2 · 2019: 5 · 2018: 6 · 2017: 1 · 2016: 6 · 2015: 4 · 2014: 8 · 2013: 8 · 2012: 5
- FWCI
- 2.46
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- American Journal of Kidney Diseases (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Nephrology › Renal Diseases and Glomerulopathies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Lipid metabolism and disorders
- Health Sciences › Medicine › Nephrology › Chronic Kidney Disease and Diabetes
- Keywords
- lipoprotein glomerulopathy, type III hyperlipoproteinemia, familial hypercholesterolemia, proteinuria, lipid metabolism disorders, renal biopsy histology
- MeSH terms
- Adult, Capillaries, Female, Humans, Hyperlipoproteinemias, Immunoglobulin G, Immunoenzyme Techniques, Kidney Diseases, Kidney Glomerulus, Lipoproteins, LDL, Middle Aged, Sclerosis, Thrombosis
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