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Idiopathic collapsing focal segmental glomerulosclerosis in an 81-year-old Japanese woman: a case report and review of the literature

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Abstract

Focal segmental glomerulosclerosis (FSGS) is classified into five variants, with the collapsing variant being the most rare. Collapsing FSGS is characterized by a black racial predominance and is often associated with human immunodeficiency virus-associated nephropathy. However, the number of idiopathic cases is increasing and the presentation of non-black patients becoming more routine. Our analysis of 15 previous reports investigating FSGS variants shows that the collapsing variant accounts for 10.6 % of FSGS cases and its average age of onset is 32 years old. The current case is one of the oldest cases of idiopathic collapsing FSGS identified, being an 81-year-old Japanese woman. She presented with severe renal insufficiency (serum creatinine 7.9 mg/dL, albumin 1.5 g/dL) and so underwent hemodialysis immediately. Urinalysis demonstrated 3+ proteinuria and 3+ hematuria and the serological work up was all negative. Renal biopsy showed wrinkling of capillary walls with collapse lumens in every glomerulus, without endothelial tubuloreticular inclusions. Combined treatment with steroids, cyclosporine and low-density lipoprotein apheresis increased urine output slightly but she was unable to withdraw from hemodialysis and died 3 months later. This variant is reported to have the highest rate of progression to end-stage renal disease, regardless of the therapeutic intervention. However, there are also examples of cases with partial or complete remission in the literature. Progressive cases, like the current case, seem to be difficult to induce remission in, so it is important to diagnose idiopathic collapsing FSGS at an early stage by performing a renal biopsy, even in elderly patients.

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References

  1. D’Agati VD, Fogo AB, Bruijn JA, Jennette JC. Pathologic classification of focal segmental glomerulosclerosis: a working proposal. Am J Kidney Dis. 2004;43:368–82.

    Article  PubMed  Google Scholar 

  2. Weiss MA, Daquioag E, Margolin EG, Pollak VE. Nephrotic syndrome, progressive irreversible renal failure, and glomerular “collapse”: a new clinicopathologic entity? Am J Kidney Dis. 1986;7:20–8.

    Article  CAS  PubMed  Google Scholar 

  3. Schwimmer JA, Markowitz GS, Valeri A, Appel GB. Collapsing glomerulopathy. Semin Nephrol. 2003;23:209–18.

    Article  PubMed  Google Scholar 

  4. Markowitz GS, Bomback AS, Perazella MA. Drug-induced glomerular disease: direct cellular injury. Clin J Am Soc Nephrol. 2015;10:1291–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Nachman PH, Jennette JC, Falk RJ. Primary glomerular disease. In: Taal MW, Chertow GM, Marsden PA, Skorecki K, Yu AS, Brenner BM, editors. Brenner & Rector’s the kidney. 9th ed. Philadelphia: Saunders; 2011. p. 1111–21.

    Google Scholar 

  6. Stokes MB, Valeri AM, Markowitz GS, D’Agati VD. Cellular focal segmental glomerulosclerosis: clinical and pathologic features. Kidney Int. 2006;70:1783–92.

    Article  CAS  PubMed  Google Scholar 

  7. Thomas DB, Franceschini N, Hogan SL, Ten Holder S, Jennette CE, Falk RJ, Jennette JC. Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants. Kidney Int. 2006;69:920–6.

    Article  CAS  PubMed  Google Scholar 

  8. Shi SF, Wang SX, Zhang YK, Zhao MH, Zou WZ. Clinicopathologic study of different variants of focal segmental glomerulosclerosis. Zhonghua Bing Li Xue Za Zhi. 2007;36:11–4.

    CAS  PubMed  Google Scholar 

  9. Deegens JK, Steenbergen EJ, Borm GF, Wetzels JF. Pathological variants of focal segmental glomerulosclerosis in an adult Dutch population—epidemiology and outcome. Nephrol Dial Transplant. 2008;23:186–92.

    Article  PubMed  Google Scholar 

  10. Niang A, Dial C, Ka EF, Lèye A, Pouye A, Ka MM, Mbengue M, Droz D, Diouf B. Nephrotic syndrome with focal and segmental glomerulosclerosis in Dakar: epidemiological and clinicopathological characteristics (about 134 cases). Dakar Med. 2008;53:45–51.

    CAS  PubMed  Google Scholar 

  11. Nada R, Kharbanda JK, Bhatti A, Minz RW, Sakhuja V, Joshi K. Primary focal segmental glomerulosclerosis in adults: is the Indian cohort different? Nephrol Dial Transplant. 2009;24:3701–7.

    Article  PubMed  Google Scholar 

  12. Taneda S, Honda K, Uchida K, Nitta K, Yumura W, Oda H, Nagata M. Histological heterogeneity of glomerular segmental lesions in focal segmental glomerulosclerosis. Int Urol Nephrol. 2012;44:183–96.

    Article  PubMed  Google Scholar 

  13. Testagrossa L, Malheiros D. Study of the morphologic variants of focal segmental glomerulosclerosis: a Brazilian report. J Bras Patol Med Lab. 2012;48:211–5.

    Article  Google Scholar 

  14. Das P, Sharma A, Gupta R, Agarwal SK, Bagga A, Dinda AK. Histomorphological classification of focal segmental glomerulosclerosis: a critical evaluation of clinical, histologic and morphometric features. Saudi J Kidney Dis Transpl. 2012;23:1008–16.

    Article  PubMed  Google Scholar 

  15. D’Agati VD, Alster JM, Jennette JC, Thomas DB, Pullman J, Savino DA, Cohen AH, Gipson DS, Gassman JJ, Radeva MK, Moxey-Mims MM, Friedman AL, Kaskel FJ, Trachtman H, Alpers CE, Fogo AB, Greene TH, Nast CC. Association of histologic variants in FSGS clinical trial with presenting features and outcomes. Clin J Am Soc Nephrol. 2013;8:399–406.

    Article  PubMed  Google Scholar 

  16. Arias LF, Jimènez CA, Arroyave MJ. Histologic variants of primary focal segmental glomerulosclerosis: presentation and outcome. J Braz Nefrol. 2013;35:112–9.

    Article  Google Scholar 

  17. Tang X, Xu F, Chen DM, Zeng CH, Liu ZH. The clinical course and long-term outcome of primary focal segmental glomerulosclerosis in Chinese adults. Clin Nephrol. 2013;80:130–9.

    Article  CAS  PubMed  Google Scholar 

  18. Shakeel S, Mubarak M, Kazi J, Jafry N, Ahmed E. Frequency and clinicopathological characteristics of variants of primary focal segmental glomerulosclerosis in adults presenting with nephrotic syndrome. J Nephropathol. 2013;2:28–35.

    Article  PubMed  PubMed Central  Google Scholar 

  19. Kwon YE, Han SH, Kie JH, An SY, Kim YL, Park KS, Nam KH, Leem AY, Oh HJ, Park JT, Chang TI, Kang EW, Kang SW, Choi KH, Lim BJ, Jeong HJ, Yoo TH. Clinical features and outcomes of focal segmental glomerulosclerosis pathologic variants in Korean adult patients. BMC Nephrol. 2014;15:52–60.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Swarnalatha G, Ram R, Ismal KM, Vali S, Sahay M, Dakshinamurty KV. Focal and segmental glomerulosclerosis: dose prognosis vary with the variants? Saudi J Kidney Dis Transpl. 2015;26:173–81.

    Article  PubMed  Google Scholar 

  21. Haas M, Spargo BH, Coventry S. Increasing incidence of focal-segmental glomerulosclerosis among adult nephropathies: a 20-year renal biopsy study. Am J Kidney Dis. 1995;26:740–50.

    Article  CAS  PubMed  Google Scholar 

  22. Grcevska L, Polenakovik M. Collapsing glomerulopathy: clinical characteristics and follow-up. Am J Kidney Dis. 1999;33:652–7.

    Article  CAS  PubMed  Google Scholar 

  23. Albaqumi M, Soos TJ, Barisoni L, Nelson PJ. Collapsing glomerulopathy. J Am Soc Nephrol. 2006;17:2854–63.

    Article  CAS  PubMed  Google Scholar 

  24. Cattran DC, Appel GB, Hebert LA, Hunsicker LG, Pohl MA, Hoy WE, Maxwell DR, Kunis CL, North America Nephrotic Syndrome Study Group. A randomized trial of cyclosporine in patients with steroid-resistant focal segmental glomerulsclerosis. Kidney Int. 1999;56:2220–6.

    Article  CAS  PubMed  Google Scholar 

  25. Miyazono M, Tomiyoshi Y, Kishi T, Ikeda Y, Sakemi T, Sanai T, Node K. A case report of nephrotic syndrome due to collapsing focal segmental glomerulosclerosis treated with low-density lipoprotein apheresis. Ther Apher Dial. 2008;12:333–6.

    Article  PubMed  Google Scholar 

  26. Muso E, Mune M, Hirano T, Hattori M, Kimura K, Watanabe T, Yokoyama H, Sato H, Uchida S, Wada T, Shoji T, Yuzawa Y, Takemura T, Sugiyama S, Nishizawa Y, Ogahara S, Yorioka N, Sakai S, Ogura Y, Yukawa S, Iino Y, Imai E, Matsuo S, Saito T. Immediate therapeutic efficacy of low-density lipoprotein apheresis for drug-resistant nephrotic syndrome: evidence from the short-term results from the POLARIS Study. Clin Exp Nephrol. 2015;19:379–86.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Nephrology, Yokohama City Minato Red Cross Hospital, 3-12-1 Shinyamashita, Naka-ku, Yokohama, Kanagawa, 231-8682, Japan

    Jun Yamazaki, Eriko Kanehisa, Wakaba Yamaguchi & Hajime Fujisawa

  2. Department of Pathology, Yokohama City Minato Red Cross Hospital, Kanagawa, Japan

    Jiro Kumagai

  3. Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan

    Kiyotaka Nagahama

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  1. Jun Yamazaki
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  2. Eriko Kanehisa
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  3. Wakaba Yamaguchi
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  4. Jiro Kumagai
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  5. Kiyotaka Nagahama
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  6. Hajime Fujisawa
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Correspondence to Jun Yamazaki.

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Yamazaki, J., Kanehisa, E., Yamaguchi, W. et al. Idiopathic collapsing focal segmental glomerulosclerosis in an 81-year-old Japanese woman: a case report and review of the literature. CEN Case Rep 5, 197–202 (2016). https://doi.org/10.1007/s13730-016-0224-3

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  • DOI: https://doi.org/10.1007/s13730-016-0224-3

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