Skip to main content

Crescentic Glomerulonephritis

  • Reference work entry
Pediatric Nephrology

Abstract

Crescentic glomerulonephritis (GN) is a rare condition in children, clinically characterized by features of rapidly progressive GN (RPGN) with rapid loss of renal function. Renal histology shows extracapillary proliferation in the Bowman’s space resulting in formation of crescents in most glomeruli. These crescents are formed when breaks in glomerular capillaries allow leakage of cells and plasma proteins into Bowman’s space, as might occur in almost any glomerulonephritis, and sometimes in diseases that do not primarily affect the glomeruli.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 369.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Jennette, JC. Rapidly progressive crescentic glomerulonephritis. Kidney Int 2003;63:1164–1177.

    Article  PubMed  Google Scholar 

  2. Morgan MD, Lorraine HL, Julie WJ, Savage C. Anti-neutrophil cytoplasm–associated glomerulonephritis. J Am Soc Nephrol 2006;17:1224–1234.

    Article  PubMed  CAS  Google Scholar 

  3. Atkins RC, Nikolic-Paterson DJ, Song Q, Lan HY. Modulators of crescentic glomerulonephritis. J Am Soc Nephrol 1996;7:2271–2278.

    PubMed  CAS  Google Scholar 

  4. Bariety J, Bruneval P, Meyrier A, Mandet C, Hill G, Jacquot C. Podocyte involvement in human immune crescentic glomerulonephritis. Kidney Int 2005;68:1109–1119.

    Article  PubMed  CAS  Google Scholar 

  5. Jennette JC, Ewert BH, Falk RJ. Do antineutrophil cytoplasmic autoantibodies cause Wegener’s granulomatosis and other forms of necrotizing vasculitis? Rheum Dis Clin North Am 1993;19:1–14.

    PubMed  CAS  Google Scholar 

  6. Ambrose L, Little M, Nourshargh S, Pusey CD. Anti-proteinase 3 antibody binding to neutrophils as demonstrated by confocal microscopy. Kidney Int 2005;68:2912–2913.

    Article  PubMed  Google Scholar 

  7. Van Rossum AP, Vandergeld YM, Limburg PC, Kallenberg CG. Human anti-neutrophil cytoplasm autoantibodies to proteinase 3 (PR3-ANCA) bind to neutrophils. Kidney Int 2005;68:537–541.

    Article  PubMed  CAS  Google Scholar 

  8. Huugen D, Tervaert JW, Heeringa P. Antineutrophil cytoplasmic autoantibodies and pathophysiology: new insights from animal models. Curr Opin Rheumatol 2004;16:4–8.

    Article  PubMed  Google Scholar 

  9. Abdel-Salam B, Iking-Konert C, Schneider M, Andrassy K, Hansch GM. Autoantibodies to neutrophil cytoplasmic antigens (ANCA) do not bind to polymorphonuclear neutrophils in blood. Kidney Int 2004;66:1009–1017.

    Article  PubMed  CAS  Google Scholar 

  10. Srivastava RN, Moudgil A, Bagga A, Vasudev AS, Bhuyan UN, Sunderam KR. Crescentic glomerulonephritis in children: a review of 43 cases. Am J Nephrol 1992;12:155–161.

    Article  PubMed  CAS  Google Scholar 

  11. El-Husseini AA, Sheashaa HA, Sabry AA, Moustafa FE, Sobh MA. Acute postinfectious crescentic glomerulonephritis: clinicopathologic presentation and risk factors. Int Urol Nephrol 2005;37:603–609.

    Article  PubMed  Google Scholar 

  12. Hoschek JC, Dreyer P, Dahal S, Walker PD. Rapidly progressive renal failure in childhood. Am J Kidney Dis 2002;40:1342–1347.

    Article  PubMed  Google Scholar 

  13. Hattori M, Kurayama H, Koitabashi Y. Japanese Society for Pediatric Nephrology. Antineutrophil cytoplasmic autoantibody-associated glomerulonephritis in children. J Am Soc Nephrol 2001;12:1493–1500.

    PubMed  CAS  Google Scholar 

  14. Hedger N, Stevens J, Drey N, Walker S, Roderick P. Incidence and outcome of pauci-immune rapidly progressive glomerulonephritis in Wessex, UK: a 10-year retrospective study. Nephrol Dial Transplant 2000;15:1593–1599.

    Article  PubMed  CAS  Google Scholar 

  15. Eisenberger U, Fakhouri F, Vanhille P et al. ANCA-negative pauci-immune renal vasculitis: histology and outcome. Nephrol Dial Transplant 2005;20:1392–1399.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  17. Ding M, Cui S, Li C et al. Loss of the tumor suppressor Vhlh leads to upregulation of Cxcr4 and rapidly progressive glomerulonephritis in mice. Nat Med 2006;12:1081–1087.

    Article  PubMed  CAS  Google Scholar 

  18. No authors listed. A clinico-pathologic study of crescentic glomerulonephritis in 50 children. A report of the Southwest Pediatric Nephrology Study Group. Kidney Int 1985;27:450–458.

    Google Scholar 

  19. Jardim HM, Leake J, Risdon RA, Barratt TM, Dillon MJ. Crescentic glomerulonephritis in children. Pediatr Nephrol 1992;6:231–235.

    Article  PubMed  CAS  Google Scholar 

  20. Niaudet P, Levy M. Glomerulonephritis à croissants diffus. In Nephrologie Pediatrique, 3rd edn. Royer P, Habib R, Mathieu H, Broyer M (eds.). Paris, Flammarion, 1983, pp. 381–394.

    Google Scholar 

  21. Dewan D, Gulati S, Sharma RK et al. Clinical spectrum and outcome of crescentic glomerulonephritis in children in developing countries. Pediatr Nephrol 2008;23:389–394.

    Article  PubMed  Google Scholar 

  22. Kashtan CE. Renal transplantation in patients with Alport syndrome. Pediatr Transplant 2006;10:651–657.

    Article  PubMed  Google Scholar 

  23. Covic A, Schiller A, Volovat C et al. Epidemiology of renal disease in Romania: a 10 year review of two regional renal biopsy databases. Nephrol Dial Transplant 2006;21:419–424.

    Article  PubMed  Google Scholar 

  24. North American Pediatric Renal Trials and Collaborative Studies. NAPRTCS 2006 Annual Report. Available: https://web.emmes.com/study/ped/annlrept/annlrept2006.

  25. Jindal KK. Management of idiopathic crescentic and diffuse proliferative glomerulonephritis: evidence-based recommendations. Kidney Int 1999;70:S33–S40.

    Article  CAS  Google Scholar 

  26. Bosch X, Guilabert A, Font J. Antineutrophil cytoplasmic antibodies. Lancet 2006;368:404–418.

    Article  PubMed  CAS  Google Scholar 

  27. Schmitt WH, van der Woude FJ. Clinical applications of antineutrophil cytoplasmic antibody testing. Curr Opin Rheumatol 2004;16:9–17.

    Article  PubMed  Google Scholar 

  28. de Groot K, Adu D, Savage CO. The value of pulse cyclophosphamide in ANCA-associated vasculitis: meta-analysis and critical review. Nephrol Dial Transplant 2001;16:2018–2027.

    Article  PubMed  CAS  Google Scholar 

  29. de Groot K, Jayne D, Tesar V, Savage C. EUVAS investigators: Randomised controlled trial of daily oral versus pulse cyclophosphamide for induction of remission in ANCA-associated systemic vasculitis. Kidney Blood Press Res 2005;28:103.

    Google Scholar 

  30. Levy JB, Turner AN, Rees AJ, Pusey CD. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med 2001;134:1033–1042.

    PubMed  CAS  Google Scholar 

  31. Gianviti A, Trompeter RS, Barratt TM, Lythgoe MF, Dillon MJ. Retrospective study of plasma exchange in patients with idiopathic rapidly progressive glomerulonephritis and vasculitis. Arch Dis Child 1996;75:186–190.

    Article  PubMed  CAS  Google Scholar 

  32. Pusey CD, Rees AJ, Evans DJ, Peters DK, Lockwood CM. Plasma exchange in focal necrotizing glomerulonephritis without anti-GBM antibodies. Kidney Int 1991;40:757–763.

    Article  PubMed  CAS  Google Scholar 

  33. Jayne DRW, Gaskin G, Rasmussen N et al. on behalf of the European Vasculitis Study Group. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 2007;18:2180–2188.

    Article  PubMed  CAS  Google Scholar 

  34. Jayne D, Rasmussen N, Andrassy K et al. European Vasculitis Study Group. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 2003;349:36–44.

    Article  PubMed  CAS  Google Scholar 

  35. Lapraik C, Watts R, Bacon P et al. on behalf of the BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis. Rheumatology 2007;46:1615–1616.

    Article  PubMed  CAS  Google Scholar 

  36. Raff A, Hebert T, Pullman J, Coco M. Crescentic post-streptococcal glomerulonephritis with nephrotic syndrome in the adult: is aggressive therapy warranted? Clin Nephrol 2005;63:375–380.

    PubMed  CAS  Google Scholar 

  37. Stassen PM, Cohen Tervaert JW, Stegeman CA. Induction of remission in active anti-neutrophil cytoplasmic antibody-associated vasculitis with mycophenolate mofetil in patients who cannot be treated with cyclophosphamide. Ann Rheum Dis 2007;66:798–802.

    Article  PubMed  CAS  Google Scholar 

  38. Hu W, Liu C, Xie H, Chen H, Zhihong LZ, Li L. Mycophenolate mofetil versus cyclophosphamide for inducing remission of ANCA vasculitis with moderate renal involvement. Nephrol Dial Transplant 2008;23:1307–1312.

    Article  PubMed  CAS  Google Scholar 

  39. Langford CA, Talar-Williams C. Mycophenolate mofetil for remission maintenance in the treatment of Wegener’s granulomatosis. Arthritis Rheum 2004;51:278–283.

    Article  PubMed  CAS  Google Scholar 

  40. Joy MS, Hogan SL, Jennette JC, Falk RJ, Nachman PH. A pilot study using mycophenolate mofetil in relapsing or resistant ANCA small vessel vasculitis. Nephrol Dial Transplant 2005;20:2725–2732.

    Article  PubMed  CAS  Google Scholar 

  41. Koukoulaki M, Jayne DR. Mycophenolate mofetil in anti-neutrophil cytoplasm antibodies-associated systemic vasculitis. Nephron Clin Pract 2006;102:c100–c107.

    Article  PubMed  CAS  Google Scholar 

  42. Rutgers A, Slot M, van Paassen P, van Breda VP, Heeringa P, Tervaert JW. Coexistence of anti-glomerular basement membrane antibodies and myeloperoxidase-ANCAs in crescentic glomerulonephritis. Am J Kidney Dis 2005;46:253–262.

    Article  PubMed  Google Scholar 

  43. Ito-Ihara T, Ono T, Nogaki F et al. Clinical efficacy of intravenous immunoglobulin for patients with MPO-ANCA-associated rapidly progressive glomerulonephritis. Nephron Clin Pract 2006;102:c35–c42.

    Article  PubMed  CAS  Google Scholar 

  44. de Groot K, Rasmussen N, Bacon PA et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 2005;52:2461–2469.

    Article  PubMed  CAS  Google Scholar 

  45. Keogh KA, Wylam ME, Stone JH, Specks U. Induction of remission by B lymphocyte depletion in eleven patients with refractory antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 2005;52:262–268.

    Article  PubMed  Google Scholar 

  46. Tam FWK. Current pharmacotherapy for the treatment of crescentic glomerulonephritis. Exper Opin Investig Drugs 2006;15:1353–1369.

    Article  CAS  Google Scholar 

  47. Booth A, Harper L, Hammad T et al. Prospective study of TNF-alpha blockade with infliximab in anti-neutrophil cytoplasmic antibody-associated systemic vasculitis. J Am Soc Nephrol 2004;15:717–721.

    Article  PubMed  CAS  Google Scholar 

  48. van Wijngaarden RAF, Hauer HA, Wolterbeek R et al. for the European Vasculitis Study Group. Clinical and histologic determinants of renal outcome in ANCA-associated vasculitis: A prospective analysis of 100 patients with severe renal involvement. J Am Soc Nephrol 2006;17:2264–2274.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2009 Springer-Verlag Berlin Heidelberg

About this entry

Cite this entry

Bagga, A. (2009). Crescentic Glomerulonephritis. In: Avner, E., Harmon, W., Niaudet, P., Yoshikawa, N. (eds) Pediatric Nephrology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-76341-3_34

Download citation

  • DOI: https://doi.org/10.1007/978-3-540-76341-3_34

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-76327-7

  • Online ISBN: 978-3-540-76341-3

  • eBook Packages: MedicineReference Module Medicine

Publish with us

Policies and ethics