nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-607Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESОпыт применения циклоспорина А в лечении идиопатического фокального сегментарного гломерулосклерозаExperience of cyclosporine А application in treatment of focal segmental glomerulosclerosisКальяноваЕ. В.KalyanovaE. V.alenakalyanova@mail.ruСтоляревичЕ. С.StolyarevichE. S.noemail@neicon.ruТомилинаН. А.TomilinaN. A.noemail@neicon.ruБирюковаЛ. С.BiryukovaL. S.noemail@neicon.ruФроловА. В.FrolovA. V.noemail@neicon.ruФедороваН. Д.FedorovaN. D.noemail@neicon.ruЗахароваЕ. В.ZakarovaE. V.noemail@neicon.ruГБУГКБ № 52 г. Москвы; ФПДО ФГОУ Московского Городского Медико-стоматологического Университета им А.И. ЕвдокимоваРоссияA.I. Evdokimov Moscow State University of Medicine and Dentistry, Chair of Nephrology; Moscow clinical hospital № 52Russian FederationГБУГКБ № 52 г. Москвы ФГБУ НЦ Трансплантологии и Искусственных органов им. академика В.И. Шумакова; ФПДО ФГОУ Московского Городского Медико-стоматологического Университета им А.И. ЕвдокимоваРоссияV.I. Shumakov Federal Research Center of Transplantology and Artificial Organs, Moscow; A.I. Evdokimov Moscow State University of Medicine and Dentistry, Chair of Nephrology; Moscow clinical hospital № 52Russian FederationГБУГКБ № 52 г. МосквыРоссияMoscow clinical hospital № 52Russian FederationГБУГКБ № 52 г. Москвы ФГБУ НЦ Трансплантологии и Искусственных органов им. академика В.И. ШумаковаРоссияV.I. Shumakov Federal Research Center of Transplantology and Artificial Organs, Moscow; Moscow clinical hospital № 52Russian FederationКафедра нефрологии ФПДО ФГОУ Московского Городского Медико-стоматологического Университета им А.И. Евдокимова; ФПДО ФГОУ Московского Городского Медико-стоматологического Университета им А.И. Евдокимова; ГБУЗ ГКБ им. С.П. БоткинаРоссияA.I. Evdokimov Moscow State University of Medicine and Dentistry, Chair of Nephrology; S.P. Botkin Moscow clinical hospitalRussian Federation201320092024154277285Copyright © Кальянова Е.В., Столяревич Е.С., Томилина Н.А., Бирюкова Л.С., Фролов А.В., Федорова Н.Д., Захарова Е.В., 20242024Кальянова Е.В., Столяревич Е.С., Томилина Н.А., Бирюкова Л.С., Фролов А.В., Федорова Н.Д., Захарова Е.В.Kalyanova E.V., Stolyarevich E.S., Tomilina N.A., Biryukova L.S., Frolov A.V., Fedorova N.D., Zakarova E.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephro.ru/jour/article/view/607

Фокальный сегментарный гломерулосклероз (ФСГС) является одним из наиболее прогностически неблагоприятных вариантов хронического гломерулонефрита. В последние десятилетия широкое распространение в лечении НС при ФСГС получил Циклоспорин А (ЦсА). Внастоящее время считается, что эффективность ЦсА при ФСГС связана с ингибицией кальцинейрин-обусловленного дефосфорилирования и уменьшением распада белка синаптоподина, результатом чего является стабилизация подоцитарного актинового цитоскелета, повреждаемого, в частности, факторами проницаемости, особенно при наличии определенной генетической предрасположенности. Ретроспективно проанализированы материалы наблюдений 49 больных с ФСГС. ЦсА применяли в 40 случаях у 36 больных (ЦсА-группа), в 24 случаях в дебюте заболевания, и в 16 – при рецидиве (4 лечились как в дебюте, так и при рецидиве). К контрольной группе отнесено 18 больных, лечившихся преднизолоном (ПЗ) или комбинацией ПЗ и циклофосфамида (ЦФ) в дебюте заболевания, причем у 5 из них при рецидиве НС применена терапия ЦсА. Результаты терапии в двух группах оказались сопоставимыми. С учетом характерного для ФСГС рецидивирующего течения, требующего длительной терапии, и значительной опасности серьезных побочных эффектов традиционно применяемых для лечения этого заболевания кортикостероидов, относительно безопасное многомесячное применение ЦсА, обеспечивающее стабильно безрецидивное течение заболевания, является в настоящее время оптимальным подходом к его терапии.

Idiopathic focal and segmental glomerulosclerosis (FSGS) is known as an unfavorable variant of glomerulonephritis. During last few decades the treatment of FSGS with Cyclosporine A (CyA) became widespread. Its efficacy is referred to inhibiting of calcineurin-dependent dephosphorylating and podocytes protein sinaptopodyn stabilization. We analyzed retrospectively the results of treatment of 49 patients with biopsy-proven FSGS. CyA was applied in 40 cases (36 patients), in 24 cases at first presentation, and in 16 – at relapse (4 patients were treated both at first and subsequent episodes). 18 patients, treated with prednisone or prednisone plus cyclophosphamide (CF) at first presentation, constituted control group, 5 of them were subsequently treated with CyA at relapse. Results of the treatment in both groups were comparable. Taking into account the relapsing course of FSGS that demands the long-term treatment with potentially serious adverse events of steroids, we conclude that relatively safe prolonged usage of CyA for sustainingremission is currently the optimal treatment option.

фокально-сегментраный гломерулосклерознефротический синдромциклоспорин Аподоцитопатиясинаптоподинfocal segmental glomerulosklerosisnephrotic syndromecyclosporine ApodocitopathysinaptopodinReferencesAppel G.B., Glassock R.J. Glomerular, vascular, and tubulointerstitial diseases // Nephrology Self-Assesment Programm. 2005. Vol. 4. P. 11–153.Appel G.B., Glassock R.J. Glomerular, vascular, and tubulointerstitial diseases // Nephrology Self-Assesment Programm. 2005. Vol. 4. P. 11–153.Appel G.B., Pollak M.R., D’Agati V. Focal and Segmental Glomerulosclerosis: Genetic and Spontaneous Causes. Comprehensive Clinical Nephrology 3rd edition. Elsevier, 2007. P. 217–231.Appel G.B., Pollak M.R., D’Agati V. Focal and Segmental Glomerulosclerosis: Genetic and Spontaneous Causes. Comprehensive Clinical Nephrology 3rd edition. Elsevier, 2007. P. 217–231.Bahiense-Oliveira M., Saldanha L.B., Andrade Mota E.L. et al. Primary glomerular disease in Brazil: 1979–1999. Is the frequency of FSGS increasing? // Clin. Nephrol. 2004. Vol. 61. P. 90–97.Bahiense-Oliveira M., Saldanha L.B., Andrade Mota E.L. et al. Primary glomerular disease in Brazil: 1979–1999. Is the frequency of FSGS increasing? // Clin. Nephrol. 2004. Vol. 61. P. 90–97.Banfi G., Moriggi M., Sabadini E. et al. The impact of prolonged immunosupression on the outcome of idiopathic focal-segmental glomerulosclerosis with nephritic syndrome in adults // Clin. Nephrol. 1991. Vol. 36 (2). P 53–59.Banfi G., Moriggi M., Sabadini E. et al. The impact of prolonged immunosupression on the outcome of idiopathic focal-segmental glomerulosclerosis with nephritic syndrome in adults // Clin. Nephrol. 1991. Vol. 36 (2). P 53–59.Beaufils H., Alphonse J.C., Guedon J. et al. Focal glomerulosclerosis: natural history and treatment. A report of 70 cases // Nephrol. 1978. Vol. 21 (2). P. 75–85.Beaufils H., Alphonse J.C., Guedon J. et al. Focal glomerulosclerosis: natural history and treatment. A report of 70 cases // Nephrol. 1978. Vol. 21 (2). P. 75–85.Braden G.L., Mulhern J.G., O’Shea M.N. et al. Changing incidence of glomerular disease in adult // Am. J. Kidney Dis. 2000. Vol. 35. P. 878–883.Braden G.L., Mulhern J.G., O’Shea M.N. et al. Changing incidence of glomerular disease in adult // Am. J. Kidney Dis. 2000. Vol. 35. P. 878–883.Braun N., Schmutzler F., Lange C. et al. Immunosupressive treatment for focal segmental glomerulosclerosis in adults (review) // Cochrane Database of systematic rewiews 2008, Issue 3. Art. No.: CD 003233. DOI:10.1002/14651858. CD003233.pub2.Braun N., Schmutzler F., Lange C. et al. Immunosupressive treatment for focal segmental glomerulosclerosis in adults (review) // Cochrane Database of systematic rewiews 2008, Issue 3. Art. No.: CD 003233. DOI:10.1002/14651858. CD003233.pub2.Cameron J.S., Turner D.R, OggC.S. et al. The long term prognosis of patient with focal segmental glomerulosclerosis // Clin. Nehrol. 1978. Vol. 10 (6). P. 213–218.Cameron J.S., Turner D.R, OggC.S. et al. The long term prognosis of patient with focal segmental glomerulosclerosis // Clin. Nehrol. 1978. Vol. 10 (6). P. 213–218.Cattran D.C. Cyclosporin in idiopathic glomerular disease associated with the nephritic syndrome: Workshop recommendations // Kidney Int. 2007. Vol. 72. P. 1429–1447.Cattran D.C. Cyclosporin in idiopathic glomerular disease associated with the nephritic syndrome: Workshop recommendations // Kidney Int. 2007. Vol. 72. P. 1429–1447.Cattran D.C., Rao P. Long-term outcome in children and adults with classic focal segmental glomerulosclerosis // Am. J. Kidney Dis. 1998. Vol. 32 (1). P. 72–79.Cattran D.C., Rao P. Long-term outcome in children and adults with classic focal segmental glomerulosclerosis // Am. J. Kidney Dis. 1998. Vol. 32 (1). P. 72–79.Cattran D.C., Appel G.B., Herbet L.A. et al. A randomized trial of cyclosporine in patients with steroid-resistant focal segmental glomerulosclerosis. North America Nephrotic Syndrome study Group // Kidney Int. 1999. Vol. 56 (6). P. 2220–2226.Cattran D.C., Appel G.B., Herbet L.A. et al. A randomized trial of cyclosporine in patients with steroid-resistant focal segmental glomerulosclerosis. North America Nephrotic Syndrome study Group // Kidney Int. 1999. Vol. 56 (6). P. 2220–2226.Chitalia V.C., Wells J.E., Robson R.A. et al. Predicting renal survival in primary focal glomerulosclerosis from the time of presentation // Kidney Int. 1999. Vol. 56 (6) P. 2236–2242.Chitalia V.C., Wells J.E., Robson R.A. et al. Predicting renal survival in primary focal glomerulosclerosis from the time of presentation // Kidney Int. 1999. Vol. 56 (6) P. 2236–2242.Chun M.J., Korbet S.M., Schwartz M.M. et al. FSGS in nephrotic adults: Presentation, prognosis, and response to therapy of the histologic variants // J. Am. Soc. Nephrol. 2004. Vol. 15. P. 2169–2177.Chun M.J., Korbet S.M., Schwartz M.M. et al. FSGS in nephrotic adults: Presentation, prognosis, and response to therapy of the histologic variants // J. Am. Soc. Nephrol. 2004. Vol. 15. P. 2169–2177.Clement L.C., Avila-Casado C., Macé C. Podocyte-secreted angiopoietin-like-4 mediates proteinuria in glucocorticoid-sensitive nephrotic syndrome // Nat. Med. 2011. Vol. 17 (1) P. 117–122.Clement L.C., Avila-Casado C., Macé C. Podocyte-secreted angiopoietin-like-4 mediates proteinuria in glucocorticoid-sensitive nephrotic syndrome // Nat. Med. 2011. Vol. 17 (1) P. 117–122.Crew R.J., Appel G.B. Focal Segmental glomerulosclerosis. The NKF Primer on Kidney Disease, 4th ed. Philadelphia. Elsevier, 2005. P. 178–182.Crew R.J., Appel G.B. Focal Segmental glomerulosclerosis. The NKF Primer on Kidney Disease, 4th ed. Philadelphia. Elsevier, 2005. P. 178–182.D’Agati V. Pathologic classification of focal segmental glomerulosclerosis // Semin. Nephrol. 2003. Vol. 23. P. 117–135.D’Agati V. Pathologic classification of focal segmental glomerulosclerosis // Semin. Nephrol. 2003. Vol. 23. P. 117–135.DantalvJ., Bigot E., Bogers W. et al. Effect of plasma protein absorbtion on protein excretion in kidney-transplant recipients with recurrent nephritic syndrome // N. Engl. J. Med. 1994. Vol. 330. P. 7–11.DantalvJ., Bigot E., Bogers W. et al. Effect of plasma protein absorbtion on protein excretion in kidney-transplant recipients with recurrent nephritic syndrome // N. Engl. J. Med. 1994. Vol. 330. P. 7–11.Deegens J.K., Steenbergen E.J., Wetzels J.F. Review on diagnosis and treatment of focal segmental glomerulosclerosis // Neth. J. Med. 2008. Vol. 66. P. 3–12.Deegens J.K., Steenbergen E.J., Wetzels J.F. Review on diagnosis and treatment of focal segmental glomerulosclerosis // Neth. J. Med. 2008. Vol. 66. P. 3–12.Ehrich J.H., Pape L., Schiffer M. Corticosterod-resistant nephritic syndrome with focal and segmental glomerulosclerosis: an update of treatment options in children // Paediatr. Drugs. 2008. Vol. 10 (1). P. 9–22.Ehrich J.H., Pape L., Schiffer M. Corticosterod-resistant nephritic syndrome with focal and segmental glomerulosclerosis: an update of treatment options in children // Paediatr. Drugs. 2008. Vol. 10 (1). P. 9–22.Faul C. The actin cytoskeleton of kidney podocytes is a direct target of the antiprotenuric effect of cyclosporine // A. Nat. Med. 2008. Vol. 14. P. 931–938.Faul C. The actin cytoskeleton of kidney podocytes is a direct target of the antiprotenuric effect of cyclosporine // A. Nat. Med. 2008. Vol. 14. P. 931–938.Faurschou M. Malignancies in Wegener’s granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients // J. Rheumatol. 2008. Vol. 35. P. 100–105.Faurschou M. Malignancies in Wegener’s granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients // J. Rheumatol. 2008. Vol. 35. P. 100–105.Garin E.H., Mu W., Arthur J.M., etal. Urinary CD80 is elevated in minimal change disease but not in focal segmental glomerulosclerosis // Kidney Int. 2010. Vol. 78 (3). P. 296–302.Garin E.H., Mu W., Arthur J.M., etal. Urinary CD80 is elevated in minimal change disease but not in focal segmental glomerulosclerosis // Kidney Int. 2010. Vol. 78 (3). P. 296–302.Genovese G, Friedman D.S., Ross M.D. et al. Assotiation of typanolytic ApoL 1 variants with kidney disease in African Americans // Science. 2010. Vol. 329. P. 841–845.Genovese G, Friedman D.S., Ross M.D. et al. Assotiation of typanolytic ApoL 1 variants with kidney disease in African Americans // Science. 2010. Vol. 329. P. 841–845.Goumenos D.S., Tsagalis G., El Nachas A.M. et al. Immunosupressive treatment of idiopathic focal segmental glomerulosklerosis: a five year follow-up study // Nephron. Clin. Pract. 2006. Vol. 104. P. 75–89.Goumenos D.S., Tsagalis G., El Nachas A.M. et al. Immunosupressive treatment of idiopathic focal segmental glomerulosklerosis: a five year follow-up study // Nephron. Clin. Pract. 2006. Vol. 104. P. 75–89.Haas M., Meehan S., Karrison T.G. et al. Changing etiologies of unexplained adult nephrotic syndrome. A comparison of renal biopsy findings from 1976–1979 and 1995–1997 // Am. J. Kidney Dis. 1997. Vol. 30. P. 621–631.Haas M., Meehan S., Karrison T.G. et al. Changing etiologies of unexplained adult nephrotic syndrome. A comparison of renal biopsy findings from 1976–1979 and 1995–1997 // Am. J. Kidney Dis. 1997. Vol. 30. P. 621–631.Hara M., Yanagihar T., Kihara I. Urinary podocytes in primary focal segmental glomerulosclerosis // Nephron. 2001. Vol. 89. P. 342–347.Hara M., Yanagihar T., Kihara I. Urinary podocytes in primary focal segmental glomerulosclerosis // Nephron. 2001. Vol. 89. P. 342–347.Ioannidis J.P. Predictors of sustained amenorrhea from pulsed intravenous cyclophosphamide in premenopausal women with systemic lupus erythematosus // J. Rheumatol. 2002. Vol. 29. P. 2129–2135.Ioannidis J.P. Predictors of sustained amenorrhea from pulsed intravenous cyclophosphamide in premenopausal women with systemic lupus erythematosus // J. Rheumatol. 2002. Vol. 29. P. 2129–2135.Kitiyakara C., Eggers P., Kopp J.B. Twenty-one year rends in ESRD due to FSGS in the United States // Am. J. Kidney Dis. 2004. Vol. 44. P. 815–825.Kitiyakara C., Eggers P., Kopp J.B. Twenty-one year rends in ESRD due to FSGS in the United States // Am. J. Kidney Dis. 2004. Vol. 44. P. 815–825.Klein M., Radhakrishnan J., Appel G.B. Cyclosporin treatment of glomerular diseases // Annu. Rev. Med. 1999. Vol. 50. P. 1–15.Klein M., Radhakrishnan J., Appel G.B. Cyclosporin treatment of glomerular diseases // Annu. Rev. Med. 1999. Vol. 50. P. 1–15.Kopp J.B., Nelson G.W., Sampath K. et al. APOL1 genetic variants in focal segmental glomerulosclerosis and HIV-associated nephropathy // J. Am. Soc. Nephrol. 2011. Vol. 22 (11). P. 2129–2137.Kopp J.B., Nelson G.W., Sampath K. et al. APOL1 genetic variants in focal segmental glomerulosclerosis and HIV-associated nephropathy // J. Am. Soc. Nephrol. 2011. Vol. 22 (11). P. 2129–2137.Korbet S.M., Schwartz M.M., Lewis E.J. Primary focal segmental glomerulosclerosis: clinical course and response to therapy // Am. J. Kidney Dis. 1994. Vol. 23 (6). P. 773–783.Korbet S.M., Schwartz M.M., Lewis E.J. Primary focal segmental glomerulosclerosis: clinical course and response to therapy // Am. J. Kidney Dis. 1994. Vol. 23 (6). P. 773–783.Korbet S.M. Treatment of focal segmental glomerulosclerosis // Kidney Int. 2002. Vol. 62. P. 2301–2310.Korbet S.M. Treatment of focal segmental glomerulosclerosis // Kidney Int. 2002. Vol. 62. P. 2301–2310.Lagrue G., Laurent J., Belghiti D. et al. Cyclosporine and idiopathic nephrotic syndrome // Lancet. 1986. Vol. 2. P. 692–693.Lagrue G., Laurent J., Belghiti D. et al. Cyclosporine and idiopathic nephrotic syndrome // Lancet. 1986. Vol. 2. P. 692–693.Lieberman K.V., Tejani A. Pediatric Nephrology Study Group: A randomized double-blind trial of cyclosporine in steroid resistant FSGS in children // J. Am. Soc. Nephrol. 1996. Vol. 7. P. 56–63.Lieberman K.V., Tejani A. Pediatric Nephrology Study Group: A randomized double-blind trial of cyclosporine in steroid resistant FSGS in children // J. Am. Soc. Nephrol. 1996. Vol. 7. P. 56–63.Maher E.R., Sweny P., Chappel M. et al. Cyclosporine in the treatment of steroid-responsive and steroid-resistant nephrotic syndrome in adults // Nephrol Dial Transplant. 1988. Vol. 3. P. 728–732.Maher E.R., Sweny P., Chappel M. et al. Cyclosporine in the treatment of steroid-responsive and steroid-resistant nephrotic syndrome in adults // Nephrol Dial Transplant. 1988. Vol. 3. P. 728–732.Matalon A., Markowitz G.S., Joseph R.E. et al. Plasmapheresis treatment of recurrent FSGS in adult transplant recipients // Clinnephrol. 2001. Vol. 56. P. 271–278.Matalon A., Markowitz G.S., Joseph R.E. et al. Plasmapheresis treatment of recurrent FSGS in adult transplant recipients // Clinnephrol. 2001. Vol. 56. P. 271–278.Matalon A., Valeri A., Appel G.B. Treatment of focal segmental glomerulosclerosis // Semin. Nephrol. 2000. Vol. 20. P. 309–317.Matalon A., Valeri A., Appel G.B. Treatment of focal segmental glomerulosclerosis // Semin. Nephrol. 2000. Vol. 20. P. 309–317.McCarthy E.T., Sharma M., Savin V.J. Circulating permeability factors in idiopathic nephrotic syndrome and focal segmental glomerulosclerosis // Clin. J. Am. Soc. Nephrol. 2010. Vol. 5 (11). P. 2115–2121.McCarthy E.T., Sharma M., Savin V.J. Circulating permeability factors in idiopathic nephrotic syndrome and focal segmental glomerulosclerosis // Clin. J. Am. Soc. Nephrol. 2010. Vol. 5 (11). P. 2115–2121.Melocoton T.L., Kamil E.S., Cohen A.H. Long-term cyclosporine A treatment in steroid-resistant and steroid-dependent nephrotic syndrome // Am. J. Kidney Dis. 1991. Vol. 18. P. 583–588.Melocoton T.L., Kamil E.S., Cohen A.H. Long-term cyclosporine A treatment in steroid-resistant and steroid-dependent nephrotic syndrome // Am. J. Kidney Dis. 1991. Vol. 18. P. 583–588.Mendoza S.A., Reznik V.M., Griswold W.R. et al. Treatment of steroid resistant focal segmental glomerulosclerosis with pulse methyl-prednisolone and alkylating agents // Pediatr. Nephrol. 1990. Vol. 4. P. 303–307.Mendoza S.A., Reznik V.M., Griswold W.R. et al. Treatment of steroid resistant focal segmental glomerulosclerosis with pulse methyl-prednisolone and alkylating agents // Pediatr. Nephrol. 1990. Vol. 4. P. 303–307.Meyrier A., Collaborativa Group of the Societ de Nephrologie. Cyclosporine in the treatment of nephrosis // Am. J. Nephrol. 1989. Vol. 9. P. S65–S71.Meyrier A., Collaborativa Group of the Societ de Nephrologie. Cyclosporine in the treatment of nephrosis // Am. J. Nephrol. 1989. Vol. 9. P. S65–S71.Meyrier A., Simon P., Perret G. et al. Remission of idiopathic nephrotic syndrome after treatment with cyclosporine // BMJ. 1986. Vol. 292. P. 789–792.Meyrier A., Simon P., Perret G. et al. Remission of idiopathic nephrotic syndrome after treatment with cyclosporine // BMJ. 1986. Vol. 292. P. 789–792.KDIGO: Clinical Practice Guideline for Glomerulonephritis, 2012.KDIGO: Clinical Practice Guideline for Glomerulonephritis, 2012.Neuhaus T.J., Burger H.R., Klingler M. et al. Long-term low-dose cyclosporin A in steroid-dependent nephrotic syndrome of childhood // Eur. J. Pediatr. 1992. Vol. 51. P. 775–778.Neuhaus T.J., Burger H.R., Klingler M. et al. Long-term low-dose cyclosporin A in steroid-dependent nephrotic syndrome of childhood // Eur. J. Pediatr. 1992. Vol. 51. P. 775–778.Niaudet P., Broyer M., Habib R. Treatment of idiopathic nephrotic syndrome with cyclosporine A in children // Clinnephrol. 1991. Vol. 35. P.S31–S36.Niaudet P., Broyer M., Habib R. Treatment of idiopathic nephrotic syndrome with cyclosporine A in children // Clinnephrol. 1991. Vol. 35. P.S31–S36.Niaudet P. French Society of Pediatric Nephrolology: Treatment of childhood steroid-resistant idiopathic nephrosis with a combination of cyclosporine and prednisone // J. Pediatr. 1994. Vol. 125. P. 981–986.Niaudet P. French Society of Pediatric Nephrolology: Treatment of childhood steroid-resistant idiopathic nephrosis with a combination of cyclosporine and prednisone // J. Pediatr. 1994. Vol. 125. P. 981–986.Pei Y., Cattran D., Delmore T. et al. Evidence suggesting under-treatment of adults with idiopathic FSGS // Am. J. Med. 1987. Vol. 82. P. 938–944.Pei Y., Cattran D., Delmore T. et al. Evidence suggesting under-treatment of adults with idiopathic FSGS // Am. J. Med. 1987. Vol. 82. P. 938–944.Ponticelli C., Glassock R.J. “Treatment of primary glomerulonephritis” Second edition. Oxford University Press,2009. P. 215–260.Ponticelli C., Glassock R.J. “Treatment of primary glomerulonephritis” Second edition. Oxford University Press,2009. P. 215–260.Ponticelli C., Passerini P. Other immunosuppressive agents for FSGS // Semin. Nephrol. 2003. Vol. 23. P. 242–248.Ponticelli C., Passerini P. Other immunosuppressive agents for FSGS // Semin. Nephrol. 2003. Vol. 23. P. 242–248.Ponticelli C., Rizzoni G., Edefonti A. et al. A randomized trial of cyclosporine in steroid-resistant idiopathic nephrotic syndrome // Kidney Int. 1993. Vol. 43 (6). P. 1377–1384.Ponticelli C., Rizzoni G., Edefonti A. et al. A randomized trial of cyclosporine in steroid-resistant idiopathic nephrotic syndrome // Kidney Int. 1993. Vol. 43 (6). P. 1377–1384.Reiser J. Induction of B7-1 inpodocytes is associated with nephrotic syndrome // J. Clin. Invest. 2004. Vol. 113 (10). P. 1390–1397.Reiser J. Induction of B7-1 inpodocytes is associated with nephrotic syndrome // J. Clin. Invest. 2004. Vol. 113 (10). P. 1390–1397.Rydel J.J., Korbet S.M., Borok R.Z. et al. Focal segmental glomerular sclerosis in adults: presentation, course, and respons to treatment // Am. J. Kidney Dis. 1995. Vol. 25 (4). P. 534–542.Rydel J.J., Korbet S.M., Borok R.Z. et al. Focal segmental glomerular sclerosis in adults: presentation, course, and respons to treatment // Am. J. Kidney Dis. 1995. Vol. 25 (4). P. 534–542.Shanklad S.J. The podocyte’s response to injury: role in proteinuria and glomerulosclerosis // Kidney International. 2006. Vol. 69. P. 2131–2147.Shanklad S.J. The podocyte’s response to injury: role in proteinuria and glomerulosclerosis // Kidney International. 2006. Vol. 69. P. 2131–2147.Tanaka R., Yoshikawa N., Kitano Y. et al. Long-term low-dose cyclosporin treatment in children with steroid-dependent nephrotic syndrome // Pediatr. Nephrol. 1993. Vol. 7. P. 249–252.Tanaka R., Yoshikawa N., Kitano Y. et al. Long-term low-dose cyclosporin treatment in children with steroid-dependent nephrotic syndrome // Pediatr. Nephrol. 1993. Vol. 7. P. 249–252.Tejani A., Suthanthiran M., Pomerantz A. A randomized controlled trial of low-dose prednisone and cyclosporine versus high-dose prednisolone in nephrotic syndrome of children // Nephron. 1991. Vol. 59. P. 96–99.Tejani A., Suthanthiran M., Pomerantz A. A randomized controlled trial of low-dose prednisone and cyclosporine versus high-dose prednisolone in nephrotic syndrome of children // Nephron. 1991. Vol. 59. P. 96–99.Troyanov S., Wall C.A., Miller J.A. et al. Toronto Glomerulonephritis Registry Group. Focal and segmental glomerulosclerosis: definition and prevalence of a partial remission // J. Am. Soc. Nephrol. 2005. Vol. 16 (4). P. 1061–1068.Troyanov S., Wall C.A., Miller J.A. et al. Toronto Glomerulonephritis Registry Group. Focal and segmental glomerulosclerosis: definition and prevalence of a partial remission // J. Am. Soc. Nephrol. 2005. Vol. 16 (4). P. 1061–1068.Tune B.M., Kirpekor R., Sibley R. et al. IV methylprednisolone and oral alkylating agent therapy of prednisolone resistant pediatric FSGS: Long-term follw up // Clin. Neprol. 1995. Vol. 43. P. 83–88.Tune B.M., Kirpekor R., Sibley R. et al. IV methylprednisolone and oral alkylating agent therapy of prednisolone resistant pediatric FSGS: Long-term follw up // Clin. Neprol. 1995. Vol. 43. P. 83–88.Velosa J.A., Donadio J.Jr., Holley K.E. Focal sclerosing glomerulopathy: a clinicopathologic study // Mayo Clin. Proc. 1975. Vol. 50 (3). P. 121–133.Velosa J.A., Donadio J.Jr., Holley K.E. Focal sclerosing glomerulopathy: a clinicopathologic study // Mayo Clin. Proc. 1975. Vol. 50 (3). P. 121–133.Webb K.L, Saragent P., Burke J.R. Cyclosporin therapy in steroid-dependent nephritic syndrome // J. Paediatr. Health. 1993. Vol. 29. P. 188–191.Webb K.L, Saragent P., Burke J.R. Cyclosporin therapy in steroid-dependent nephritic syndrome // J. Paediatr. Health. 1993. Vol. 29. P. 188–191.Wehrmann M., Bohle A., Held H. et al. Long-term prognosis of focal sclerosing glomerulonephritis An analysis of 250 cases with particular regard to tubulointerstitial changes // Clin. Nephrol. 1990. Vol. 33 (3). P. 115–122.Wehrmann M., Bohle A., Held H. et al. Long-term prognosis of focal sclerosing glomerulonephritis An analysis of 250 cases with particular regard to tubulointerstitial changes // Clin. Nephrol. 1990. Vol. 33 (3). P. 115–122.Wei C., El Hindi S., Li J., Fornoni A. et al. Circulating urokinase receptor as a cause of focal segmental glomerulosclerosis // Nat. Med. 2011. Vol. 17 (8). P. 952–960.Wei C., El Hindi S., Li J., Fornoni A. et al. Circulating urokinase receptor as a cause of focal segmental glomerulosclerosis // Nat. Med. 2011. Vol. 17 (8). P. 952–960.Wiggins R.C. The spectrum of podocytopathies: A unifying view of glomerular diseases // Kidney International. 2007. Vol. 71. P. 1205–1214.Wiggins R.C. The spectrum of podocytopathies: A unifying view of glomerular diseases // Kidney International. 2007. Vol. 71. P. 1205–1214.

The authors declare that there are no conflicts of interest present.