Skip to main content

Advertisement

SpringerLink
Log in

Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis

  • Original Article
  • Published:
Pediatric Nephrology Aims and scope Submit manuscript

Abstract

Background

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a small- and medium-sized vasculitis classically seen in adult patients, with peak onset near the fifth to seventh decade of life. There is little data on ANCA-associated vasculitis in pediatric patients, and most studies have limited follow-up.

Methods

This is a retrospective chart review of 22 patients with ANCA-positive glomerulonephritis in a single institution from 1991 to 2013.

Results

Of the 22 patients, eight (36 %) required renal replacement therapy (RRT) at diagnosis; four of these patients recovered sufficient renal function to initially discontinue dialysis. Five patients (23 %) were treated with plasmapheresis at presentation. The median time from presentation until first clinical or serologic relapse was 1.7 ± 1.2 years. After a median follow-up of 5.8 years, just over half of our patients had chronic kidney disease (CKD) stages 1–3 (55 %). Seven (32 %) patients progressed to end-stage renal disease (ESRD) and eventually required kidney transplant.

Conclusion

ANCA-associated glomerulonephritis is a rare disorder in children. Presentation and outcomes vary significantly among patients. More research is required to follow these patients who are diagnosed in childhood to further characterize the long-term outcome of the disease.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

Abbreviations

AAV:

ANCA-associated vasculitis

ANCA:

Antineutrophil cytoplasmic antibody

AGN:

ANCA-associated glomerulonephritis classification

c-ANCA:

Cytoplasmic antineutrophil cytoplasmic antibody

CKD:

Chronic kidney disease

ESRD:

End-stage renal disease

EPA:

Eosinophilic granulomatosis with polyangiitis

GFR:

Glomerular filtration rate

GPA:

Granulomatosis with polyangiitis

MPA:

Microscopic polyangiitis

MPO:

Myeloperoxidase

PR3:

Proteinase 3

p-ANCA:

Perinuclear antineutrophil cytoplasmic antibody

RPGN:

Rapidly progressing glomerulonephritis

RRT:

Renal replacement therapy

References

  1. Jennette JC, Falk RJ (1990) Antineutrophil cytoplasmic autoantibodies and associated diseases: a review. Am J Kidney Dis 6:517–529

    Article  Google Scholar 

  2. Kallenberg CG, Stegeman CA, Abdulahad WH, Heeringa P (2013) Pathogenesis of ANCA-associated vasculitis: new possibilities for intervention. Am J Kidney Dis 6:1176–1187

    Article  Google Scholar 

  3. Kamesh L, Harper L, Savage CO (2002) ANCA-positive vasculitis. J Am Soc Nephrol 7:1953–1960

    Article  Google Scholar 

  4. Bohm M, Gonzalez Fernandez MI, Ozen S, Pistorio A, Dolezalova P, Brogan P, Barbano G, Sengler C, Klein-Gitelman M, Quartier P, Fasth A, Herlin T, Terreri MT, Nielsen S, van Rossum MA, Avcin T, Castell ER, Foeldvari I, Foell D, Kondi A, Kone-Paut I, Kuester RM, Michels H, Wulffraat N, Amer HB, Malattia C, Martini A, Ruperto N (2014) Clinical features of childhood granulomatosis with polyangiitis (wegener’s granulomatosis). Pediatr Rheum Online J 12:18

    Article  Google Scholar 

  5. Hattori M, Kurayama H, Koitabashi Y, Japanese Society for Pediatric Nephrology (2001) Antineutrophil cytoplasmic autoantibody-associated glomerulonephritis in children. J Am Soc Nephrol 7:1493–1500

    Google Scholar 

  6. Peco-Antic A, Bonaci-Nikolic B, Basta-Jovanovic G, Kostic M, Markovic-Lipkovski J, Nikolic M, Spasojevic B (2006) Childhood microscopic polyangiitis associated with MPO-ANCA. Pediatr Nephrol 1:46–53

    Article  Google Scholar 

  7. Schwartz GJ, Haycock GB, Edelmann CM Jr, Spitzer A (1976) A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 2:259–263

    Google Scholar 

  8. Berden AE, Ferrario F, Hagen EC, Jayne DR, Jennette JC, Joh K, Neumann I, Noel LH, Pusey CD, Waldherr R, Bruijn JA, Bajema IM (2010) Histopathologic classification of ANCA-associated glomerulonephritis. J Am Soc Nephrol 10:1628–1636

    Article  Google Scholar 

  9. Ford SL, Polkinghorne KR, Longano A, Dowling J, Dayan S, Kerr PG, Holdsworth SR, Kitching AR, Summers SA (2014) Histopathologic and clinical predictors of kidney outcomes in ANCA-associated vasculitis. Am J Kidney Dis 2:227–235

    Article  Google Scholar 

  10. Noone DG, Twilt M, Hayes WN, Thorner PS, Benseler S, Laxer RM, Parekh RS, Hebert D (2014) The new histopathologic classification of ANCA-associated GN and its association with renal outcomes in childhood. Clin J Am Soc Nephrol 10:1684–1691

    Article  Google Scholar 

  11. Belostotsky VM, Shah V, Dillon MJ (2002) Clinical features in 17 paediatric patients with Wegener granulomatosis. Pediatr Nephrol 9:754–761

    Article  Google Scholar 

  12. Arulkumaran N, Jawad S, Smith SW, Harper L, Brogan P, Pusey CD, Salama AD (2011) Long-term outcome of paediatric patients with ANCA vasculitis. Pediatr Rheum Online J 9:12

    Article  Google Scholar 

  13. Siomou E, Tramma D, Bowen C, Milford DV (2012) ANCA-associated glomerulonephritis/systemic vasculitis in childhood: clinical features-outcome. Pediatr Nephrol 10:1911–1920

    Article  Google Scholar 

  14. Yu F, Huang JP, Zou WZ, Zhao MH (2006) The clinical features of anti-neutrophil cytoplasmic antibody-associated systemic vasculitis in Chinese children. Pediatr Nephrol 4:497–502

    Article  Google Scholar 

  15. de Joode AA, Sanders JS, Stegeman CA (2013) Renal survival in proteinase 3 and myeloperoxidase ANCA-associated systemic vasculitis. Clin J Am Soc Nephrol 10:1709–1717

    Article  Google Scholar 

  16. Sinico RA, Di Toma L, Radice A (2013) Renal involvement in anti-neutrophil cytoplasmic autoantibody associated vasculitis. Autoimmun Rev 4:477–482

    Article  Google Scholar 

  17. Krmar RT, Kagebrand M, Hansson ME, Halling SE, Asling-Monemi K, Herthelius M, Holtback U, Christensson M, Wernerson A, Bruchfeld A (2013) Renal-limited vasculitis in children: a single-center retrospective long-term follow-up analysis. Clin Nephrol 5:388–394

    Article  Google Scholar 

  18. Akikusa JD, Schneider R, Harvey EA, Hebert D, Thorner PS, Laxer RM, Silverman ED (2007) Clinical features and outcome of pediatric Wegener’s granulomatosis. Arthritis Rheum 5:837–844

    Article  Google Scholar 

  19. Basu B, Mahapatra TK, Mondal N (2015) Favourable renal survival in paediatric microscopic polyangiitis: efficacy of a novel treatment algorithm. Nephrol Dial Transplant 30(Suppl 1):i113–i118

    PubMed  Google Scholar 

  20. Sacri AS, Chambaraud T, Ranchin B, Florkin B, See H, Decramer S, Flodrops H, Ulinski T, Allain-Launay E, Boyer O, Dunand O, Fischbach M, Hachulla E, Pietrement C, Le Pogamp P, Stephan JL, Belot A, Nivet H, Nobili F, Guillevin L, Quartier P, Deschenes G, Salomon R, Essig M, Harambat J (2015) Clinical characteristics and outcomes of childhood-onset ANCA-associated vasculitis: a French nationwide study. Nephrol Dial Transplant 30(Suppl 1):i104–i112

    PubMed  Google Scholar 

  21. Valentini RP, Smoyer WE, Sedman AB, Kershaw DB, Gregory MJ, Bunchman TE (1998) Outcome of antineutrophil cytoplasmic autoantibodies-positive glomerulonephritis and vasculitis in children: a single-center experience. J Pediatr 2:325–328

    Article  Google Scholar 

  22. Walsh M, Flossmann O, Berden A, Westman K, Hoglund P, Stegeman C, Jayne D, European Vasculitis Study Group (2012) Risk factors for relapse of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 2:542–548

    Article  Google Scholar 

  23. Ellis EN, Wood EG, Berry P (1995) Spectrum of disease associated with anti-neutrophil cytoplasmic autoantibodies in pediatric patients. J Pediatr 1:40–43

    Article  Google Scholar 

  24. Day CJ, Howie AJ, Nightingale P, Shabir S, Adu D, Savage CO, Hewins P (2010) Prediction of ESRD in pauci-immune necrotizing glomerulonephritis: quantitative histomorphometric assessment and serum creatinine. Am J Kidney Dis 2:250–258

    Article  Google Scholar 

  25. Bakkaloglu A, Ozen S, Baskin E, Besbas N, Gur-Guven A, Kasapcopur O, Tinaztepe K (2001) The significance of antineutrophil cytoplasmic antibody in microscopic polyangitis and classic polyarteritis nodosa. Arch Dis Child 5:427–430

    Article  Google Scholar 

  26. Cabral DA, Uribe AG, Benseler S, O’Neil KM, Hashkes PJ, Higgins G, Zeft AS, Lovell DJ, Kingsbury DJ, Stevens A, McCurdy D, Chira P, Abramson L, Arkachaisri T, Campillo S, Eberhard A, Hersh AO, Huber AM, Kim S, Klein-Gitelman M, Levy DM, Li SC, Mason T, Dewitt EM, Muscal E, Nassi L, Reiff A, Schikler K, Singer NG, Wahezi D, Woodward A, ARChiVe (A Registry for Childhood Vasculitis: e-entry) Investigators Network (2009) Classification, presentation, and initial treatment of Wegener’s granulomatosis in childhood. Arthritis Rheum 11:3413–3424

    Article  Google Scholar 

  27. Khalighi MA, Wang S, Henriksen KJ, Bock M, Keswani M, Chang A, Meehan SM (2015) Pauci-immune glomerulonephritis in children: a clinicopathologic study of 21 patients. Pediatr Nephrol 6:953–959

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatric Nephrology, Indiana University School of Medicine, 699 Riley Hospital Drive, Rm 230, Indianapolis, IN, 46202, USA

    Anne M. Kouri & Sharon P. Andreoli

Authors
  1. Anne M. Kouri
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sharon P. Andreoli
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Anne M. Kouri.

Ethics declarations

Conflict of interest

The authors have no conflicts of interest to disclose.

Ethics

This study was approved by the Institutional Review Board governing Indiana University School of Medicine. A waiver of consent was granted for this study as this was approved as an IRB-exempt study.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Kouri, A.M., Andreoli, S.P. Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis. Pediatr Nephrol 32, 449–455 (2017). https://doi.org/10.1007/s00467-016-3490-6

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00467-016-3490-6

Keywords

Search

Navigation