Pediatric Nephrology

, Volume 27, Issue 10, pp 1911–1920 | Cite as

ANCA-associated glomerulonephritis/systemic vasculitis in childhood: clinical features–outcome

  • Ekaterini Siomou
  • Despoina Tramma
  • Claire Bowen
  • David V. Milford
Original Article

Abstract

Background

Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis and systemic vasculitis (AAGNV) is uncommon in childhood.

Methods

This is a retrospective study of AAGNV cases diagnosed over a 13-year period in a tertiary pediatric nephrology department.

Results

Thirteen cases of AAGNV were identified: seven Wegener granulomatosis (WG) and six microscopic polyangiitis (MPA). Acute renal failure/nephrotic range proteinuria (NRP) was found in 77 % of the patients (4 with WG, all with MPA). Eleven (85 %) patients showed necrotizing glomerulonephritis (NGN), with ≥50 % crescents identified in nine patients (69 %) (4 with WG, 5 with MPA). Treatment with methylprednisolone, cyclophosphamide and plasma exchange resulted in extra-renal remission and antibody reduction in all patients and renal function improvement/stabilization in 77 % of the patients. Three patients, all without oliguria at presentation and few sclerotic lesions, had normal renal function at follow-up. Chronic kidney disease (CKD) stages 2 and 3–4 were observed in four (WG) and three (MPA) patients, respectively. Three patients (23 %) developed end stage renal disease: two were MPA patients with severe presentation (markedly impaired glomerular filtration rate, oliguria, NRP, crescentic NGN, glomerular sclerosis) and one was a WG patient with extensive interstitial fibrosis/tubular atrophy.

Conclusions

Severe renal involvement was more common in children with MPA than WG. Treatment with methylprednisolone, cyclophosphamide and plasma exchange induced extra-renal remission/serological response and renal function improvement/stabilization. Markedly decreased GFR, oliguria, NRP, and chronic glomerular lesions at presentation were predictors of poor outcome.

Keywords

ANCA-associated vasculitis Wegener granulomatosis Microscopic polyangiitis Necrotizing glomerulonephritis Childhood 

Abbreviations

AAGNV

ANCA-associated glomerulonephritis and systemic vasculitis

ANCA

Antineutrophil cytoplasmic antibody

c-ANCA

cytoplasmic ANCA

CKD

Chronic kidney disease

CYCP

Cyclophosphamide

eGFR

estimated glomerular filtration rate

ELISA

Enzyme-linked immunosorbent assay

ESRD

End stage renal disease

MP

Methylprednisolone

MPA

Microscopic polyangiitis

MPO

Myeloperoxidase

NCGN

Necrotizing crescentic glomerulonephritis

NGN

Necrotizing glomerulonephritis

NRP

Nephrotic range proteinuria

p-ANCA

perinuclear ANCA

PR3

Proteinase 3

UPr:UCr

Urine protein to creatinine ratio

WG

Wegener granulomatosis

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Copyright information

© IPNA 2012

Authors and Affiliations

  • Ekaterini Siomou
    • 1
    • 3
  • Despoina Tramma
    • 1
  • Claire Bowen
    • 2
  • David V. Milford
    • 1
  1. 1.Department of NephrologyBirmingham Children’s Hospital NHS Foundation TrustBirminghamUK
  2. 2.Department of Paediatric PathologyBirmingham Children’s Hospital NHS Foundation TrustBirminghamUK
  3. 3.University Hospital of IoanninaIoanninaGreece

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