The problem of focal-segmental glomerulosclerosis in primary glomerulonephritis
https://doi.org/10.36485/1561-6274-2023-27-1-108-114
Abstract
The concept of focal segmental glomerulosclerosis in clinical practice is used to describe both a separate disease and morphological characteristics of secondary kidney damage. Most often, focal segmental glomerulosclerosis occurs as a result of the course of primary glomerulonephritis and is the cause of the development of nephrotic syndrome. A feature of the course of nephrotic syndrome in focal segmental glomerulosclerosis is a longer response to steroid therapy, or the formation of dependence/resistance to glucocorticosteroids. Despite the development of scientific progress, the problem of focal segmental glomerulosclerosis in primary glomerulonephritis is still relevant. Understanding the mechanisms of podocyte damage and disease progression is important not only for basic research, but also for everyday clinical practice.
Keywords
About the Authors
S. L. MorozovRussian Federation
Sergey L. Morozov
Research Clinical Institute of Pediatrics named after acad. Yu. E. Veltishcheva
Faculty of Pediatrics
Department of Hospital Pediatrics No. 2
Moscow
V. V. Dlin
Russian Federation
Vladimir V. Dlin
Research Clinical Institute of Pediatrics named after acad. Yu. E. Veltishcheva
Moscow
References
1. Fogo A. B. Causes and pathogenesis of focal segmental glomerulosclerosis. Nat Rev Nephrol 2015; 11 (2): 76–87. doi: 10.1038/nrneph.2014.216
2. Klembovsky V. I., Dlin V. V., Osmanov I. M., Morozov S. L. Clinical morphology of kidney disease in children. Electronic edition. 2011
3. Collins A. J., Foley R. N., Herzog C., Chavers B., Gilbertson D., Ishani A. et al. US Renal Data System 2010 Annual Data Report. Am J Kidney Dis Off J Natl Kidney Found 2011; 57 (1 Suppl 1): A8, e1-526. doi: 10.1053/j.ajkd.2010.10.007
4. Hogan J., Radhakrishnan J. The treatment of minimal change disease in adults. J Am Soc Nephrol JASN 2013; 24 (5): 702–711. doi: 10.1681/ASN.2012070734
5. D’Agati V. D., Kaskel F. J., Falk R. J. Focal segmental glomerulosclerosis. N Engl J Med 2011; 365 (25): 2398–2411. doi:10.1056/NEJMra1106556
6. Morozov S. L., Dlin V. V. Markers of steroid resistance of the primary nephrotic syndrome in children. Practical medicine 2021; 19 (1): 15–21. (In Russ.) doi: 10.32000/2072-1757-2021-1-15-21 EDN GLNTLJ
7. Ignatova M. S, Dlin V. V. Nephrotic syndrome: past, present and future. Russian Bulletin of Perinatology and Pediatrics 2017; 62 (6): 29–44. (In Russ.) URL: https://www.ped-perinatology.ru/jour/article/view/588
8. Klembovsky A. I. Dysplastic bases for the development of childhood nephropathies. 1989; 8 (1): 53–60 (In Russ.)
9. Klembovsky A. I. Clinical morphology of nephropathy. In: Pediatric Nephrology. Ed. Ignatova MS, Veltishcheva Yu. E. Medicine, Leningrad, 1989; 27–59
10. D’Agati V. D., Fogo A. B., Bruijn J. A., Jennette J. C. Pathologic classification of focal segmental glomerulosclerosis: a working proposal. Am J. Kidney Dis Off J Natl Kidney Found 2004; 43 (2): 368–382. doi:10.1053/j.ajkd.2003.10.024
11. D’Agati V. D., Alster J. M., Jennette J. C., Thomas D. B., Pullman J., Savino D. A. et al. Association of histologic variants in FSGS clinical trial with presenting features and outcomes. Clin J Am Soc Nephrol CJASN 2013; 8 (3): 399–406. doi:10.2215/CJN.06100612
12. Rossini M., Fogo A. B. Interpreting segmental glomerular sclerosis. Curr Diagn Pathol [Internet] 2004; 10 (1): 1–10. doi:10.1016/j.cdip.2003.09.001. Available from: https://www.diagnostichistopathology.co.uk/article/S0968-6053(03)00073-5/fulltext
13. Braun F., Homeyer I., Alachkar N., Huber T. B. Immunemediated entities of (primary) focal segmental glomerulosclerosis. Cell Tissue Res 2021; 385 (2): 423–434. doi: 10.1007/s00441-021-03454-3
14. Le Berre L., Hervé C., Buzelin F., Usal C., Soulillou J.-P., Dantal J. Renal macrophage activation and Th2 polarization precedes the development of nephrotic syndrome in Buffalo / Mna rats. Kidney Int 2005; 68 (5): 2079–2090. doi: 10.1111/j.1523-1755.2005.00664.x
15. Steinmetz O. M., Summers S. A., Gan P.-Y., Semple T., Holdsworth S. R., Kitching A. R. The Th17-defining transcription factor RORγt promotes glomerulonephritis. J Am Soc Nephrol JASN 2011; 22 (3): 472–483. doi:10.1681/ASN.2010040435
16. Shao X. S., Yang X. Q., Zhao X. D., Li Q., Xie Y. Y., Wang X. G. et al. The prevalence of Th17 cells and FOXP3 regulate T cells (Treg) in children with primary nephrotic syndrome. Pediatr Nephrol Berl Ger 2009; 24 (9): 1683–1690. doi: 10.1007/s00467-009-1194-x
17. Benz K., Büttner M., Dittrich K., Campean V., Dötsch J., Amann K. Characterisation of renal immune cell infiltrates in children with nephrotic syndrome. Pediatr Nephrol Berl Ger 2010; 25 (7): 1291–1298. doi: 10.1007/s00467-010-1507-0
18. Fornoni A., Sageshima J., Wei C., Merscher-Gomez S., Aguillon-Prada R., Jauregui A. N. et al. Rituximab targets podocytes in recurrent focal segmental glomerulosclerosis. Sci Transl Med 2011; 3 (85): 85ra46. doi: 10.1126/scitranslmed.3002231
19. Gallon L., Leventhal J., Skaro A., Kanwar Y., Alvarado A. Resolution of Recurrent Focal Segmental Glomerulosclerosis after Retransplantation. N Engl J Med [Internet] 2012; 366 (17): 1648–1649. doi: 10.1056/NEJMc1202500. Available from: http://www.nejm.org/doi/abs/10.1056/NEJMc1202500
20. Desmedt S., Desmedt V., Delanghe J. R., Speeckaert R., Speeckaert M. M. The intriguing role of soluble urokinase receptor in inflammatory diseases. Crit Rev Clin Lab Sci [Internet] 2017; 54 (2): 117–133. doi: 10.1080/10408363.2016.1269310. Available from: https://www.tandfonline.com/doi/full/10.1080/10408363.2016.1269310
21. Xu Y., Berrou J., Chen X., Fouqueray B., Callard P., Sraer J.-D. et al. Induction of Urokinase Receptor Expression in Nephrotoxic Nephritis. Nephron Exp Nephrol [Internet] 2001; 9 (6): 397–404. doi: 10.1159/000052638. Available from: https://www.karger.com/Article/FullText/52638
22. Wei C., Möller C. C., Altintas M. M., Li J., Schwarz K., Zacchigna S. et al. Modification of kidney barrier function by the urokinase receptor. Nat Med [Internet] 2008; 14 (1): 55–63. doi: 10.1038/nm1696. Available from: http://www.nature.com/articles/nm1696
23. Wei C., Trachtman H., Li J., Dong C., Friedman A. L., Gassman J. J. et al. Circulating suPAR in Two Cohorts of Primary FSGS. J Am Soc Nephrol [Internet] 2012; 23 (12): 2051–2059. doi: 10.1681/ASN.2012030302. Available from: https://pubmed.ncbi.nlm.nih.gov/23138488/
24. Möller C. C., Flesche J., Reiser J. Sensitizing the Slit Diaphragm with TRPC6 Ion Channels. J Am Soc Nephrol [Internet] 2009; 20 (5): 950–953. doi: 10.1681/ASN.2008030329. Available from: https://jasn.asnjournals.org/lookup/doi/10.1681/ASN.2008030329
25. Morozov S. L., Dlin V. V. The role of cation channels of the potential TRPC receptor in the pathogenesis of idiopathic nephrotic syndrome in children. Russian Bulletin of Perinatology and Pediatrics 2021; 66 (5): 67–74. (In Russ.) doi: 10.21508/1027-4065-2021-66-5-67-74
26. Krall P., Canales C. P., Kairath P., Carmona-Mora P., Molina J., Carpio J. D. et al. Podocyte-Specific Overexpression of Wild Type or Mutant Trpc6 in Mice Is Sufficient to Cause Glomerular Disease. PLoS ONE [Internet] 2010; 5 (9): e12859. doi: 10.1371/journal.pone.0012859. Available from: https://dx.plos.org/10.1371/journal.pone.0012859
27. Meyrier A. Mechanisms of Disease: focal segmental glomerulosclerosis. Nat Clin Pract Nephrol [Internet] 2005; 1 (1): 44–54. doi: 10.1038/ncpneph0025. Available from: http://www.nature.com/articles/ncpneph0025
28. Appel D., Kershaw D. B., Smeets B., Yuan G., Fuss A., Frye B. et al. Recruitment of podocytes from glomerular parietal epithelial cells. J Am Soc Nephrol JASN 2009; 20 (2): 333–343. doi:10.1681/ASN.2008070795
29. Smeets B., Stucker F., Wetzels J., Brocheriou I., Ronco P., Gröne H.-J. et al. Detection of activated parietal epithelial cells on the glomerular tuft distinguishes early focal segmental glomerulosclerosis from minimal change disease. Am J Pathol 2014; 184 (12): 3239–3248. doi: 10.1016/j.ajpath.2014.08.007
Review
For citations:
Morozov S.L., Dlin V.V. The problem of focal-segmental glomerulosclerosis in primary glomerulonephritis. Nephrology (Saint-Petersburg). 2023;27(1):108-114. (In Russ.) https://doi.org/10.36485/1561-6274-2023-27-1-108-114