Improvement of nephrotic syndrome by intensive lipid-lowering therapy in a patient with lipoprotein glomerulopathy
Abstract
Lipoprotein glomerulopathy (LPG) is a rare hereditary disease characterized by the accumulation of much thrombi material consisting of lipoproteins at the glomerular capillary lumen. Most patients show nephrotic syndrome; nearly half progress to chronic renal failure. Intensive therapy with lipid-lowering agents reportedly engenders clinical remission with histological resolution. We report the case of a 14-year-old Japanese female patient who had been in a nephrotic condition with hematuria from 4 years old and who had been diagnosed based on pathological and molecular examination at 7 years old. We initially treated the patient with probucol, enalapril (angiotensin-converting enzyme inhibitor: ACEI), and dipyridamole from age 7, but achieved no improvement in her nephrotic status. Subsequently, we replaced probucol with bezafibrate at age 11 and added atorvastatin calcium hydrate and valsartan (angiotensin II receptor blocker: ARB) the following year. The next 3 years' treatment improved her nephrotic status, decreased serum apolipoprotein E, and markedly decreased intraglomerular lipoprotein thrombi. Early and intensive therapy with antilipidemic drugs combined with ACEI and ARB is inferred to be effective for LPG.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
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?
Additional treatments including renin-angiotensin-aldosterone system inhibitors and plasma adsorption/exchange therapy have been proven effective in LPG monitoring.
“Additional treatments include renin-angiotensin-aldosterone system inhibitors and plasma adsorption/exchange therapy have also been proven to be effective in LPG monitoring [21, 22].”
From A case report of youth-onset lipoprotein glomerulopathy with APOE Chicago mutation (2024) · cites this paper as [21]
Fibrates and ACEI/ARB may be effective for LPG remission.
“Though no effective treatment has been established, fibrates and ACEI/ARB may be effective for LPG remission (Arai et al., 2003; Ieiri, Hotta, & Taguma, 2003; Matsunaga et al., 2009).”
From Clinical and genetic analysis of lipoprotein glomerulopathy patients caused by <i>APOE</i> mutations (2020) · cites this paper as Matsunaga et al., 2009
Two case studies and one small population study showed that lipid-lowering therapies are linked to improved renal status in LPG patients.
“Two case studies and one small population study showed that lipid-lowering therapies are linked to improved renal status in LPG patients (27)(28)(29).”
From Macrophage-derived apoESendai suppresses atherosclerosis while causing lipoprotein glomerulopathy in hyperlipidemic mice (2014) · cites this paper as [28]
Worldwide, 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 [49]
Previous case reports found combined treatment with glucocorticoids and immunosuppressants ineffective; intensified treatment with lipid-lowering drugs including fibrates provided clinical and histological relief, with serum cholesterol, triglycerides, and ApoE decreased, and consecutive renal biopsies demonstrated complete disappearance of lipoprotein thrombi.
“Previous case reports that found combined treatment with glucocorticoids and immunosuppressants ineffective used intensified treatment with lipid-lowering drugs (including fibrates) to provide clinical and histological relief for patients. In those cases, serum cholesterol, triglycerides, and ApoE decreased, and consecutive renal biopsies demonstrated complete disappearance of lipoprotein thrombi.”
From A boy and his mother with lipoprotein glomerulopathy: Two case reports and literature review (2025) · cites this paper as [34]
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
Arai, T., Yamashita, S., Yamane, M., Manabe, N., Matsuzaki, T., Kiriyama, K., Kanayama, Y., Himeno, S., & Matsuzawa, Y. (2003) · Atherosclerosis · doi:10.1016/s0021-9150(03)00194-1 · Cited by 43 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
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-citationTsimihodimos, V., & Elisaf, M. (2011) · Current Opinion in Lipidology · doi:10.1097/mol.0b013e328345ebb0 · Cited by 33 · see citing works
Wu, H., Yang, Y., & Hu, Z. (2018) · Journal of Atherosclerosis and Thrombosis · doi:10.5551/jat.41996 · Cited by 24 · see citing works
Tramontano, 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
A founder haplotype of APOE-Sendai mutation associated with lipoprotein glomerulopathy
Toyota, K., Hashimoto, T., Ogino, D., Matsunaga, A., Ito, M., Masakane, I., Degawa, N., Sato, H., Shirai, S., Umetsu, K., et al. (2013) · Journal of Human Genetics · doi:10.1038/jhg.2013.8 · Cited by 19 · see citing works
Self-citationClinical 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
Common Apolipoprotein E Gene Mutations Contribute to Lipoprotein Glomerulopathy in China
Han, J., Pan, Y., Chen, Y., Li, X., Xing, G., Shi, J., Hou, P., Zhang, H., & Wang, H. (2010) · Nephron Clinical Practice · doi:10.1159/000276578 · Cited by 13 · see citing works
Tavori, H., Fan, D., Giunzioni, I., Zhu, L., Linton, M. F., Fogo, A. B., & Fazio, S. (2014) · Journal of Lipid Research · doi:10.1194/jlr.m049874 · Cited by 10 · see citing works
Lipoprotein 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
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
A boy and his mother with lipoprotein glomerulopathy: Two case reports and literature review
Zhang, S., Fan, L., Li, Z., Liu, T., Jing, X., & Guo, Q. (2025) · Medicine · doi:10.1097/md.0000000000041628 · Cited by 2 · see citing works
Identification of a founder haplotype of <i>APOE Kyoto</i> variant in lipoprotein glomerulopathy
Wu, H., He, Z., Yang, J., Zhou, Y., Yang, Z., Hu, Z., & Yang, Y. (2025) · Clinical Kidney Journal · doi:10.1093/ckj/sfaf234
- Authors
- Matsunaga, A., Furuyama, M., Hashimoto, T., Toyoda, K., Ogino, D., & Hayasaka, K.
- Affiliations
- Yamagata University (JP)
- Corresponding author
- Matsunaga, Akira
- ORCID
- Hayasaka, Kiyoshi
- Venue
- Clinical and Experimental Nephrology
- Published
- 2009-12
- Volume
- 13
- Issue
- 6
- Pages
- 659-662
- ISSN
- 1342-1751 (ISSN-L)
- 1437-7799
- OpenAlex
- W2051182555
- Cited by
- 28
- References
- 2
- Citations by year
- 2026: 1 · 2025: 3 · 2024: 2 · 2023: 1 · 2022: 2 · 2021: 1 · 2020: 1 · 2019: 4 · 2018: 4 · 2016: 1 · 2014: 1 · 2013: 3 · 2012: 1
- FWCI
- 0.92
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Clinical and Experimental Nephrology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Nephrology › Renal Diseases and Glomerulopathies
- Health Sciences › Medicine › Nephrology › Chronic Kidney Disease and Diabetes
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Lipid metabolism and disorders
- Keywords
- lipoprotein glomerulopathy, nephrotic syndrome, lipid-lowering therapy, atorvastatin, bezafibrate, valsartan, enalapril, probucol, apolipoprotein E, chronic kidney disease, pediatric patients
- MeSH terms
- Valsartan, Adolescent, Angiotensin-Converting Enzyme Inhibitors, Hypolipidemic Agents, Apolipoproteins E, Bezafibrate, Child, Child, Preschool, Enalapril, Female, Hematuria, Humans, Kidney Diseases, Kidney Failure, Chronic, Kidney Glomerulus, Nephrotic Syndrome, Probucol, Tetrazoles, Valine, Angiotensin II Type 1 Receptor Blockers
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
