A Case of Lipoprotein Glomerulopathy with apoE Chicago and apoE (Glu3Lys) Treated with Fenofibrate
Open accessAbstract
Lipoprotein glomerulopathy (LPG) is characterized by the accumulation of lipoprotein thrombi within glomerular capillaries. This rare disorder is associated with various types of mutations in the apolipoprotein E gene (apoE). Herein, we present a case of LPG with a combination of apoE Chicago (Arg147Pro) and apoE (Glu3Lys) mutations. A 51-year-old Japanese woman presented with severe (3+) proteinuria. The initial renal biopsy showed glomerular capillary dilation and occlusion with lipid granules, a specific characteristic of LPG. Phenotype, genotype, and apoE DNA sequence analyses detected 2 mutations as described above within the same allele. Although both mutations had already been reported in 1 case of LPG each, this is the first time that the combination of the 2 mutations was identified in the same case. Familial analysis detected the same mutations in the patient’s mother. However, she has not suffered LPG thus far. In addition, a re-analysis of the previous LPG case with apoE (Glu3Lys) also identified the apoE Chicago mutation, as was observed in our case. Treatment with fenofibrate and irbesartan was initiated, and urinary protein excretion ceased within 1 year; recurrence was not observed after an additional 2 years of follow-up. A second biopsy after 2 years showed great improvement, with lipoprotein thrombi identified only in 2 of 18 glomeruli.
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
APOE Kyoto was also reported as a compound heterozygote of p.Arg50His (Arg32His) and APOE Hong Kong (Asp230Tyr), while APOE Chicago was also reported as a compound heterozygote of APOE5 (Glu3Lys).
“APOE Kyoto was also reported as a compound heterozygote of p.Arg50His (Arg32His)88) and APOE Hong Kong (Asp230Tyr)89), while APOE Chicago was also reported as a compound heterozygote of APOE5 (Glu3Lys)90).”
From Impact of Apolipoprotein E Variants: A Review of Naturally Occurring Variants and Clinical Features (2025) · cites this paper as [90]
The citation to this paper is attached to the APOE Chicago/APOE5 clause. The Discussion states that apoE Chicago (Arg147Pro) and apoE5 (Glu3Lys) were on the same allele, with wild-type E3 on the other—not on separate alleles as a compound heterozygote would require. (LLM, conf=0.90)
Checked by a reasoning model — may be wrong.
Experimental — accuracy not yet measuredWorldwide, only 22 cases of LPG including this case have reported the results of more than one native kidney biopsy.
“Worldwide, only 22 cases of LPG including this case have reported the results of more than one native kidney biopsy.14,15,29,32,44–49”
From Lipoprotein Glomerulopathy, First Case Report from Canada (2022) · cites this paper as [15]
The Case Report (Pathological Findings and Treatment and Prognosis; pages not provided) describes an initial renal biopsy in 2011 and a second in 2013, supporting this paper as an example of an LPG case with more than one biopsy. It does not report a worldwide total of 22 such cases; the number 22 in the second-biopsy passage refers to glomeruli in the specimen. (LLM, conf=0.86)
Experimental — accuracy not yet measuredNephropathy associated with apoE5 (Glu3Lys) alone has not yet been reported.
“Nephropathy associated with apoE5 (Glu3Lys) alone has not yet been reported.”
From Focal segmental glomerulosclerosis with heterozygous apolipoprotein E5 (Glu3Lys) (2018) · cites this paper as [4]
A few cases of LPG associated with apoE5 (Glu3Lys) have been reported.
“A few cases of LPG associated with apoE5 (Glu3Lys) have been reported”
From Focal segmental glomerulosclerosis with heterozygous apolipoprotein E5 (Glu3Lys) (2018) · cites this paper as [4]
In reported LPG cases with apoE5, other LPG-specific apoE variants (apoE-Chicago, apoE-Sendai) were also detected.
“but apoE variants specific to LPG, e.g., apoE-Chicago [4] and apoE-Sendai [5], were also detected in these cases”
From Focal segmental glomerulosclerosis with heterozygous apolipoprotein E5 (Glu3Lys) (2018) · cites this paper as [4]
The specific role of apoE5 (Glu3Lys) in LPG is unknown.
“the specific role of apoE (Glu3Lys) is unknown.”
From Focal segmental glomerulosclerosis with heterozygous apolipoprotein E5 (Glu3Lys) (2018) · cites this paper as [4]
APOE-Chicago was first reported in a male Mexican-American patient and later in a Japanese female patient.
“APOE‐Chicago was also first reported in Chinese LPG patients which was first reported in a male Mexican‐American patient (Sam et al., 2006) and later in a Japanese female patient (Kodera et al., 2017).”
From Clinical and genetic analysis of lipoprotein glomerulopathy patients caused by <i>APOE</i> mutations (2020) · cites this paper as Kodera et al., 2017
Case 2, the mother of Case 1, had both apoE-Chicago and apoE5 (Glu3Lys) but did not develop LPG; she had apoE4 on the other allele.
“Case 2 in Table 1, the mother of Case 1, showed both apoE-Chicago and apoE5 (Glu3Lys) as in Case 1, but nevertheless did not develop LPG.She had an isoform apoE4 on the other allele unlike Case 1.”
From A case of lipoprotein glomerulopathy with a rare apolipoprotein E isoform combined with neurofibromatosis type I (2018) · cites this paper as [5]
Only two cases of LPG with apoE5 (Glu3Lys) have been reported, and they were confirmed to have apoE-Chicago.
“To the best of our knowledge, only two cases of LPG with apoE5 (Glu3Lys) have been reported [5,6] and these are confirmed to have apoE-Chicago by Kodera [5] (Table 1).”
From A case of lipoprotein glomerulopathy with a rare apolipoprotein E isoform combined with neurofibromatosis type I (2018) · cites this paper as [5]
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Cites
Lipoprotein Glomerulopathy: Glomerular Lipoprotein Thrombi in a Patient With Hyperlipoproteinemia
Saito, T., Sato, H., Kudo, K.-I., Oikawa, S.-I., Shibata, T., Hara, Y., Yoshinaga, K., & Sakaguchi, H. (1989) · American Journal of Kidney Diseases · doi:10.1016/s0272-6386(89)80134-9 · Cited by 148 · see citing works
Self-citationGeorgiadou, D., Stamatakis, K., Efthimiadou, E. K., Kordas, G., Gantz, D., Chroni, A., & Stratikos, E. (2013) · Journal of Lipid Research · doi:10.1194/jlr.m030965 · Cited by 37 · see citing works
Flagged first, then by citations
Research works printed but not linked: 14
- [2]Saito T Matsunaga A Ito K Nakashima H Topics in lipoprotein glomerulopathy: an overview. Clin Exp Nephrol 2014 18 214 217 24149835
- [3]Sam R Wu H Yue L Mazzone T Schwartz MM Arruda JA Dunea G Singh AK Lipoprotein glomerulopathy: a new apolipoprotein E mutation with enhanced glomerular binding. Am J Kidney Dis 2006 47 539 548 16490634
- [4]Dong LM Yamamura T Yamamoto A Enhanced binding activity of an apolipoprotein E mutant, APO E5, to LDL receptors on human fibroblasts. Biochem Biophys Res Commun 1990 168 409 414 2334412
- [5]Wardell MR Rall SC Jr Schaefer EJ Kane JP Weisgraber KH Two apolipoprotein E5 variants illustrate the importance of the position of additional positive charge on receptor-binding activity. J Lipid Res 1991 32 521 528 1648586
- [6]Kobayashi J Shirai K Murano T Misawa Y Tashiro J Yoshida T Shinomiya M A case of hyperlipidemia with homozygous apolipoprotein E5 (Glu3–>gt;Lys). Biochim Biophys Acta 2002 1583 117 121 12069856
- [7]Miyata T Sugiyama S Nangaku M Suzuki D Uragami K Inagi R Sakai H Kurokawa K Apolipoprotein E2/E5 variants in lipoprotein glomerulopathy recurred in transplanted kidney. J Am Soc Nephrol 1999 10 1590 1595 10405216
- [8]Saito T Matsunaga A Oikawa S Impact of lipoprotein glomerulopathy on the relationship between lipids and renal diseases. Am J Kidney Dis 2006 47 199 211 16431249
- [9]Oikawa S Matsunaga A Saito T Sato H Seki T Hoshi K Hayasaka K Kotake H Midorikawa H Sekikawa A Hara S Abe K Toyota T Jingami H Nakamura H Sasaki J Apolipoprotein E Sendai (Arginine 145->Proline): a new variant associated with lipoprotein glomerulopathy. J Am Soc Nephrol 1997 8 820 823 9176854
- [10]Matsunaga A Sasaki J Komatsu T Tsuji E Moriyama K Koga T Arakawa K Oikawa S Saito T Kita T Doi T A novel apolipoprotein E mutation, E2 (Arg25Cys), in lipoprotein glomerulopathy. Kidney Int 1999 56 421 427 10432380
- [11]Watanabe Y Ozaki I Yoshida F Fukatsu A Itoh Y Matsuo S Sakamoto N A case of nephrotic syndrome with glomerular lipoprotein deposition with capillary ballooning and mesangiolysis. Nephron 1989 51 265 270 2644575
- [12]Hoffmann M Scharnagl H Panagiotou E Banghard W Wieland H März W Diminished LDL receptor and high heparin binding of apolipoprotein E2 Sendai associated with lipoprotein glomerulopathy. J Am Soc Nephrol 2001 12 524 530 11181800
- [13]Saito T Matsunaga A Ito K Nakashima H Topics in lipoprotein glomerulopathy: an review. Clin Exp Nephrol 2014 18 214 217 24149835
- [15]Toyota K Hashimoto T Ogino D Matsunaga A Ito M Masakane I Degawa N Sato H Shirai S Umetsu K Tamiya G Saito T Hayasaka K A founder haplotype of APOE-Sendai mutation associated with lipoprotein glomerulopathy. J Hum Genet 2013 58 254 258 23407349
- [16]Hu Z Huang S Wu Y Liu Y Liu X Su D Tao Y Fu P Zhang X Peng Z Zhang S Yang Y Hereditary features, treatment, and prognosis of the lipoprotein glomerulopathy in patients with the APOE Kyoto mutation. Kidney Int 2014 85 416 424 24025644
Cited by
Sorted by citations
APOE gene variants in primary dyslipidemia
Khalil, Y. A., Rabès, J.-P., Boileau, C., & Varret, M. (2021) · Atherosclerosis · doi:10.1016/j.atherosclerosis.2021.05.007 · Cited by 170 · see citing works
Apolipoprotein E–related glomerular disorders
Saito, T., Matsunaga, A., Fukunaga, M., Nagahama, K., Hara, S., & Muso, E. (2020) · Kidney International · doi:10.1016/j.kint.2019.10.031 · Cited by 77 · see citing works
Self-citationTramontano, D., D'Erasmo, L., Larouche, M., Brisson, D., Lauzière, A., Di Costanzo, A., Bini, S., Minicocci, I., Covino, S., Baratta, F., et al. (2025) · Atherosclerosis · doi:10.1016/j.atherosclerosis.2025.119146 · Cited by 23 · see citing works
Clinical and genetic analysis of lipoprotein glomerulopathy patients caused by <i>APOE</i> mutations
Yang, M., Weng, Q., Pan, X., Hussain, H. M. J., Yu, S., Xu, J., Yu, X., Liu, Y., Jin, Y., Zhang, C., et al. (2020) · Molecular Genetics & Genomic Medicine · doi:10.1002/mgg3.1281 · Cited by 15 · see citing works
Impact of Apolipoprotein E Variants: A Review of Naturally Occurring Variants and Clinical Features
Matsunaga, A., & Saito, T. (2025) · Journal of Atherosclerosis and Thrombosis · doi:10.5551/jat.65393 · Cited by 14 · see citing works
Self-citationFocal segmental glomerulosclerosis with heterozygous apolipoprotein E5 (Glu3Lys)
Sasaki, M., Yasuno, T., Ito, K., Matsunaga, A., Hisano, S., Abe, Y., Miyake, K., Masutani, K., Nakashima, H., & Saito, T. (2018) · CEN Case Reports · doi:10.1007/s13730-018-0331-4 · Cited by 9 · see citing works
Self-citationLipoprotein Glomerulopathy, First Case Report from Canada
Ting, J. A., McRae, S. A., Schwartz, D., Barbour, S. J., & Riazy, M. (2022) · International Journal of Nephrology and Renovascular Disease · doi:10.2147/ijnrd.s364890 · Cited by 7 · see citing works
Takasaki, S., Matsunaga, A., Joh, K., & Saito, T. (2018) · CEN Case Reports · doi:10.1007/s13730-018-0309-2 · Cited by 7 · see citing works
Self-citationWang, R., Zhao, C., Chen, W., Liu, Z., & Xie, F. (2022) · Journal of Medical Case Reports · doi:10.1186/s13256-022-03302-0 · Cited by 5 · see citing works
A case report of youth-onset lipoprotein glomerulopathy with APOE Chicago mutation
Liu, Y., Cheng, Y., Wen, Y., Li, C., Chen, G., & Li, M. (2024) · BMC Nephrology · doi:10.1186/s12882-024-03515-z · Cited by 2 · see citing works
- Authors
- Kodera, H., Mizutani, Y., Sugiyama, S., Miyata, T., Ehara, T., Matsunaga, A., & Saito, T.
- Affiliations
- Yokkaichi Social Insurance Hospital (JP)
- United Centers for Advanced Research and Translational Medicine, Tohoku University (JP)
- Matsumoto University (JP)
- Fukuoka University (JP)
- Corresponding author
- Kodera, Hitoshi
- Venue
- Case Reports in Nephrology and Dialysis
- Publisher
- S. Karger AG
- Published
- 2017-07-27
- Volume
- 7
- Issue
- 2
- Pages
- 112-120
- ISSN
- 2296-9705 (ISSN-L)
- PMC
- PMC5618396
- OpenAlex
- W2741532658
- Cited by
- 17
- References
- 2
- Citations by year
- 2025: 3 · 2024: 1 · 2022: 3 · 2021: 2 · 2020: 2 · 2019: 2 · 2018: 4
- FWCI
- 1.96
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- Case Reports in Nephrology and Dialysis (publishedVersion, cc-by-nc)
- PubMed Central (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- 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, apolipoprotein E, compound heterozygosity, proteinuria, fenofibrate, irbesartan, familial analysis, genotype-phenotype correlation
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
