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A case of a 6-year-old girl with anti-neutrophil cytoplasmic autoantibody-negative pauci-immune crescentic glomerulonephritis

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Abstract

A 6-year-old girl was admitted to our hospital with proteinuria, hematuria, skin rash and joint pain of the lower limbs. Due to rapid progression of renal insufficiency, hemodialysis and peritoneal dialysis were performed. She was diagnosed with rapidly progressive glomerulonephritis. Kidney biopsy showed severe crescent formation (50% of glomeruli) and no deposition of any immunoglobulins or complements. Serologically, anti-neutrophil cytoplasmic autoantibody (ANCA) was negative not only by ELISA against proteinase-3 and myeloperoxidase-ANCA but also by indirect immunofluorescent assay against cytoplasmic and perinuclear ANCA. Anti-glomerular basement membrane antibody was also negative. In the acute phase, proinflammatory cytokines such as soluble tumor necrosis factor receptor 1 (sTNFR1), soluble interleukin (IL)-2 receptor (sIL2R), IL-6 and chemokine IL-8 were elevated. The patient was diagnosed with ANCA-negative pauci-immune crescentic glomerulonephritis (CrGN). Intensive treatment with methylprednisolone pulse therapy, plasma exchange, and multiple drug therapy including prednisolone and cyclophosphamide resulted in histopathological improvement and complete remission of proteinuria. There was a possibility that sTNFR1, sIL2R, IL-6 and IL-8 might be involved in the initiation and progression of ANCA-negative pauci-immune CrGN, and to remove and suppress these cytokines might be an effective way to treat ANCA-negative pauci-immune CrGN.

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References

  1. Couser WG. Rapidly progressive glomerulonephritis: classification, pathogenic mechanisms, and therapy. Am J Kidney Dis. 1998;6:449–64.

    Google Scholar 

  2. Bolton WK. Rapidly progressive glomerulonephritis. Semin Nephrol. 1996;6:517–26.

    Google Scholar 

  3. Morgan MD, Harper L, Williams J, Savage C. Anti-neutrophil cytoplasm-associated glomerulonephritis. J Am Soc Nephrol. 2006;17:1224–34.

    Article  CAS  PubMed  Google Scholar 

  4. Chen M, Yu F, Wang SX, Zou WZ, Zhao MH, Wang HY. Antineutrophil cytoplasmic autoantibody-negative pauci-immune crescentic glomerulonephritis. J Am Soc Nephrol. 2007;18:599–605.

    Article  PubMed  Google Scholar 

  5. Hung PH, Chiu YL, Lin WC, Chiang WC, Chen YM, Lin SL, et al. Poor renal outcome of antineutrophil cytoplasmic antibody negative pauci-immune glomerulonephritis in Taiwanese. J Formos Med Assoc. 2006;10:804–12.

    Article  Google Scholar 

  6. Eisenberger U, Fakhouri F, Vanhille P, Beaufils H, Mahr A, Guillevin L, et al. ANCA-negative pauci-immune renal vasculitis: histology and outcome. Nephrol Dial Transplant. 2005;20:1392–9.

    Article  PubMed  Google Scholar 

  7. Harada T, Uzu T, Namba T, Yamamoto R, Takahara K, Yamauchi A. ANCA-negative pauci-immune crescentic glomerulonephritis complicated with recurrent massive gastrointestinal hemorrhage. Clin Exp Nephrol. 2005;9:174–8.

    Article  PubMed  Google Scholar 

  8. Chow KM, Wang AY, Mac-Moune Lai F, Wong TY, Li PK. Rapid recurrence of ANCA-negative pauci-immune vasculitis after cadaveric renal transplantation. Am J Kidney Dis. 2001;1:E3.

    Google Scholar 

  9. Fukuda Y, Watanabe H, Ohtomo Y, Yabuta K. Immunologically mediated chronic tubulo-interstitial nephritis caused by valproate therapy. Nephron. 1996;72:328–9.

    Article  CAS  PubMed  Google Scholar 

  10. Ackoundou-N’guessan C, Canaud B, Leray-Moragues H, Droz D, Baldet P, Pages M. Collapsing focal segmental glomerulosclerosis as a possible complication of valproic acid. S Afr Med J. 2007;97:388–90.

    PubMed  Google Scholar 

  11. Falk RJ, Terrell RS, Charles LA, Jennette JC. Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro. Proc Natl Acad Sci USA. 1990;87:4115–9.

    Article  CAS  PubMed  Google Scholar 

  12. Arimura Y, Minoshima S, Kamiya Y, Tanaka U, Nakabayashi K, Kitamoto K, et al. Serum myeloperoxidase and serum cytokine in anti-myeloperoxidase antibody-associated glomerulonephritis. Clin Nephrol. 1993;5:256–64.

    Google Scholar 

  13. Condliffe AM, Chilvers ER, Haslett C, Dransfield I. Priming differentially regulates neutrophil adhesion molecule expression/function. Immunology. 1996;89:105–11.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Matsumoto K. Spontaneous release of interleukin-8 by glomerular macrophages from patients with rapidly progressive crescentic glomerulonephritis. Nephrol Dial Transplant. 1994;9:1693.

    PubMed  CAS  Google Scholar 

  15. Ralston DR, Marsh CB, Lowe MP, Wewers MD. Antineutrophil cytoplasmic antibodies induce monocyte IL-8 release. J Clin Invest. 1997;100:1416–24.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Zhou Z, Richard C, Menard HA. De novo synthesis of proteinase 3 by cytokine primed circulating human polymorphonuclear neutrophils and mononuclear cells. J Rheumatol. 2000;10:2406–11.

    Google Scholar 

  17. Cockwell P, Brooks CJ, Adu D, Savage C. Interleukin-8: a pathogenetic role in antineutrophil cytoplasmic autoantibody-associated glomerulonephritis. Kidney Int. 1999;55:852–63.

    Article  CAS  PubMed  Google Scholar 

Download references

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Authors and Affiliations

  1. Department of Pediatrics, Takamatsu Red Cross Hospital, 4-1-3 Ban-cho, Takamatsu, Kagawa, 760-0017, Japan

    Maki Shimizu, Takanori Sekiguchi, Natsuko Kishi, Aya Goji, Tomoko Takahashi, Hiroko Kozan, Zenichi Sakaguchi & Katsuaki Ohara

  2. Department of Pediatrics, The Institute of Health Bioscience, The University of Tokushima Graduate School, Tokushima, Japan

    Yukiko Kinoshita, Sato Matsuura, Kenichi Suga, Maki Urushihara, Shuji Kondo & Shoji Kagami

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  1. Maki Shimizu
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  2. Takanori Sekiguchi
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  3. Natsuko Kishi
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  4. Aya Goji
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  5. Tomoko Takahashi
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  7. Zenichi Sakaguchi
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  8. Yukiko Kinoshita
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  13. Shoji Kagami
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  14. Katsuaki Ohara
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Correspondence to Maki Shimizu.

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Shimizu, M., Sekiguchi, T., Kishi, N. et al. A case of a 6-year-old girl with anti-neutrophil cytoplasmic autoantibody-negative pauci-immune crescentic glomerulonephritis. Clin Exp Nephrol 15, 596–601 (2011). https://doi.org/10.1007/s10157-011-0423-3

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  • DOI: https://doi.org/10.1007/s10157-011-0423-3

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