Top

Pediatric Nephrology

Published in:

01-03-2017 | Original Article

Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis

Authors: Anne M. Kouri, Sharon P. Andreoli

Published in: Pediatric Nephrology | Issue 3/2017

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.

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

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

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

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 12:18CrossRef

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

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 1:46–53CrossRef

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

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 10:1628–1636CrossRef

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 2:227–235CrossRef

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 10:1684–1691CrossRef

11.

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

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 9:12CrossRef

13.

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

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 4:497–502CrossRef

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 10:1709–1717CrossRef

16.

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

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 5:388–394CrossRef

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 5:837–844CrossRef

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–i118PubMed

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–i112PubMed

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 2:325–328CrossRef

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 2:542–548CrossRef

23.

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

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 2:250–258CrossRef

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 5:427–430CrossRef

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 11:3413–3424CrossRef

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 6:953–959CrossRef

Title: Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis
Authors: Anne M. Kouri
Sharon P. Andreoli
Publication date: 01-03-2017
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Nephrology / Issue 3/2017
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-016-3490-6

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis

Abstract

Background

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Methods

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 3/2017

A rare cause of pyrexia in a transplant patient: Questions

Favorable renal outcome in Japanese children with ANCA-associated pauci-immune glomerulonephritis: impact of urine screening program

Fatal disseminated cowpox virus infection in an adolescent renal transplant recipient

A rare cause of pyrexia in a transplant patient: Answers

Predictive ability of urinary biomarkers for outcome in children with acute kidney injury

A malfunctioning peritoneal dialysis catheter: Questions