Remission induced by renal protective therapy in nephrotic syndrome with thin basement membrane in an older patient: a case report.
Dapagliflozin
Enalapril
Ezetimibe
Nephrotic syndrome
Remission
Rosuvastatin
Thin glomerular basement membrane
Journal
Journal of medical case reports
ISSN: 1752-1947
Titre abrégé: J Med Case Rep
Pays: England
ID NLM: 101293382
Informations de publication
Date de publication:
03 May 2024
03 May 2024
Historique:
received:
29
11
2023
accepted:
13
04
2024
medline:
4
5
2024
pubmed:
4
5
2024
entrez:
3
5
2024
Statut:
epublish
Résumé
Adult nephrotic syndrome is a well-known kidney disease that causes heavy proteinuria, hypoalbuminemia, hypercholesterolemia, edema, and hypertension. The treatment varies according to its underlying cause but often faces medication resistance or adverse drug effects. A Japanese woman in her 80s presented with nephrotic syndrome after a 3 year latent period of urinary protein and occult blood. She did not have any secondary causes of nephrotic syndrome. Renal biopsy revealed thin glomerular basement membrane, partial foot process fusion on electron microscopy with minor glomerular change on light microscopy, and slight coarse immunoglobulin M deposition in the mesangium on immunofluorescence microscopy, which was inconsistent with any other glomerular diseases. Without steroid treatment, she dramatically remitted from proteinuria after the administration of the renal protective agents enalapril, ezetimibe, rosuvastatin, and dapagliflozin. Recurrence after 8 months of follow-up subsided with the administration of additional doses of the agents. This case illustrated the novel outcomes of combining medical treatment without steroid use for nephrotic syndrome with thin glomerular basement membrane disease. At the time of writing this report, the patient's renal function was stable and she was free of edema, although moderate proteinuria and occult hematuria persisted. The final diagnosis was uncertain because of the lack of genetic investigation; however, the response to the aforementioned medical treatment suggests the effectiveness of the supportive therapy.
Sections du résumé
BACKGROUND
BACKGROUND
Adult nephrotic syndrome is a well-known kidney disease that causes heavy proteinuria, hypoalbuminemia, hypercholesterolemia, edema, and hypertension. The treatment varies according to its underlying cause but often faces medication resistance or adverse drug effects.
CASE PRESENTATION
METHODS
A Japanese woman in her 80s presented with nephrotic syndrome after a 3 year latent period of urinary protein and occult blood. She did not have any secondary causes of nephrotic syndrome. Renal biopsy revealed thin glomerular basement membrane, partial foot process fusion on electron microscopy with minor glomerular change on light microscopy, and slight coarse immunoglobulin M deposition in the mesangium on immunofluorescence microscopy, which was inconsistent with any other glomerular diseases. Without steroid treatment, she dramatically remitted from proteinuria after the administration of the renal protective agents enalapril, ezetimibe, rosuvastatin, and dapagliflozin. Recurrence after 8 months of follow-up subsided with the administration of additional doses of the agents.
CONCLUSIONS
CONCLUSIONS
This case illustrated the novel outcomes of combining medical treatment without steroid use for nephrotic syndrome with thin glomerular basement membrane disease. At the time of writing this report, the patient's renal function was stable and she was free of edema, although moderate proteinuria and occult hematuria persisted. The final diagnosis was uncertain because of the lack of genetic investigation; however, the response to the aforementioned medical treatment suggests the effectiveness of the supportive therapy.
Identifiants
pubmed: 38702831
doi: 10.1186/s13256-024-04564-6
pii: 10.1186/s13256-024-04564-6
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
237Informations de copyright
© 2024. The Author(s).
Références
Hull RP, Goldsmith DJA. Nephrotic syndrome in adults. BMJ. 2008;336:1185–9.
doi: 10.1136/bmj.39576.709711.80
pubmed: 18497417
pmcid: 2394708
Suryawanshi M, Karnik S, Roy S. Clinicopathological analysis of glomerular disease of adult onset nephrotic syndrome in an Indian cohort—a retrospective study. J Clin Diagn Res. 2017;11:25–30.
Uzzo M, Moroni G, Ponticelli C. Thin basement membrane: an underrated cause of end-stage renal disease. Nephron. 2023. https://doi.org/10.1159/000528243 .
doi: 10.1159/000528243
pubmed: 36882005
Hamilton P, Myers J, Gillham J, Ayers G, Brown N, Venning M. Urinary protein selectivity in nephrotic syndrome and pregnancy: resurrection of a biomarker when renal biopsy is contraindicated. Clin Kidney J. 2014;7(6):595–8.
doi: 10.1093/ckj/sfu103
pubmed: 25859379
pmcid: 4389140
Hommos MS, Zeng C, Liu Z, Troost JP, Rosenberg AZ, Palmer M, et al. Global glomerulosclerosis with nephrotic syndrome; the clinical importance of age adjustment. Kidney Int. 2018;93:1175–82.
doi: 10.1016/j.kint.2017.09.028
pubmed: 29273332
Mubarak M, Kazi JI. IgM nephropathy revisited. Nephrourol Mon. 2012;4:603–8.
doi: 10.5812/numonthly.2805
pubmed: 23573499
pmcid: 3614302
Kfoury H, Arafah M. The pathological spectrum associated with the ultrastructural finding of thin glomerular basement membrane: a tertiary medical city experience and review of the literature. Ultrastruct Pathol. 2017;41:51–4.
doi: 10.1080/01913123.2016.1258021
pubmed: 28029267
Cosio FG, Falkenhain ME, Sedmak DD. Association of thin glomerular basement membrane with other glomerulopathies. Kidney Int. 1994;46:471–4.
doi: 10.1038/ki.1994.296
pubmed: 7967360
Fervenza FC, Sethi S. Focal segmental glomerulosclerosis: clinical features and diagnosis. In: Glassock RJ, Rovin BH, editors. UpToDate. Waltham: UpToDate Inc; 2023.
Savige J, Lipska-Zietkiewicz BS, Watson E, Hertz JM, Deltas C, Mari F, et al. Guidelines for genetic testing and management of Alport syndrome. Clin J Am Soc Nephrol. 2022;17:143–54. Erratum in: Clin J Am Soc Nephrol. 2023;18:510.
Rusu E-E, Zilisteanu D-S, Ciobotaru L-M, Gherghiceanu M, Procop A, Jurcut RO, et al. The impact of kidney biopsy for Fabry nephropathy evaluation on patients’ management and long-term outcomes: experience of a single center. Biomedicines. 2022;10:1520.
doi: 10.3390/biomedicines10071520
pubmed: 35884826
pmcid: 9313342
Kondo M, Mori T, Oshita T, Ohashi A, Sohara E, Uchida S, et al. Case of hereditary kidney disease presenting thin basement membrane with a single heterozygous variant of Intersectin 2. J Rural Med. 2023;18:143–8.
doi: 10.2185/jrm.2022-048
pubmed: 37032986
pmcid: 10079461
Pusey CD, Segelmark M. Anti-GBM (Goodpasture) disease: pathogenesis, clinical manifestations, and diagnosis. In: Glassock RJ, Fervenza FC, editors. UpToDate. Waltham: UpToDate Inc; 2023.
Gansevoort RT, Sluiter WJ, Hemmelder MH, de Zeeuw D, de Jong PE. Antiproteinuric effect of blood-pressure-lowering agents: a meta- analysis of comparative trials. Nephrol Dial Transplant. 1995;10:1963–74.
pubmed: 8643149
The GISEN Group (Gruppo Italiano di Studi Epidemiologici in Nefrologia). Randomised controlled trial of effect of ramipril on decline in glomerular filtration rate and risk of terminal renal failure in proteinuric, non diabetic nephropathy. Lancet. 1997;349:1857–63.
doi: 10.1016/S0140-6736(96)11445-8
Vaziri ND, Anzalone DA, Catini J. Statins in Chronic Kidney Disease: when and when not to use them. J Fam Pract. 2016;65: supp_az_0816.
pubmed: 27660844
Kong X, Yuan H, Fan J, Li Z, Wu T, Jiang L. Lipid-lowering agents for nephrotic syndrome. Cochrane Database Syst Rev. 2013;12: CD005425.
Murashima R, Sai E, Tagawa Y, Yanagawa H, Ishiwata S, Kawaguchi Y, et al. Usefulness of dapagliflozin for nephrotic syndrome secondary to diabetic kidney disease. Intern Med. 2022;61:3699–702.
doi: 10.2169/internalmedicine.9121-21
pubmed: 35466166
pmcid: 9841097
Heerspink HJL, Stefánsson BV, Correa-Rotter R, Chertow GM, Greene T, Hou FF, et al. Dapagliflozin in patients with chronic kidney disease. N Engl J Med. 2020;383:1436–46.
doi: 10.1056/NEJMoa2024816
pubmed: 32970396
Ogawa R, Miyoshi K, Nagao T, Jotoku M, Irita J, Okura T, et al. Ultrastructure of glomerular podocyts in the incipient phase of minimal change nephrotic syndrome with thin basement membrane disease. Nihon Jinzo Gakkai Shi. 2012;54:1192–6.
pubmed: 23387282
Chiba Y, Nagasawa T, Kin S, Takahashi K, Yoshida M, Oe Y, et al. Spontaneous remission of minimal change nephrotic syndrome in an elderly man. CEN Case Rep. 2021;10:301–7.
doi: 10.1007/s13730-020-00554-x
pubmed: 33398783
pmcid: 8019442
van Paassen P, van Breda Vriesman PJ, van Rie H, Tervaert JW. Signs and symptoms of thin basement membrane nephropathy: a prospective regional study on primary glomerular disease—The Limburg Renal Registry. Kidney Int. 2004;66:909–13.
doi: 10.1111/j.1523-1755.2004.00835.x
pubmed: 15327380
Matthaiou A, Poulli T, Deltas C. Prevalence of clinical, pathological and molecular features of glomerular basement membrane nephropathy caused by COL4A3 or COL4A4 mutations: a systematic review. Clin Kidney J. 2020;13:1025–36.
doi: 10.1093/ckj/sfz176
pubmed: 33391746
pmcid: 7769542
Kamiar A, Alitter Q, Capcha JMC, Saad A, Webster KA, Shehadeh LA. Ascending aortic aneurysm and histopathology in Alport syndrome: a case report. BMC Nephrol. 2023;24:1–6.
doi: 10.1186/s12882-023-03345-5