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.
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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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Siomou, E., Tramma, D., Bowen, C. et al. ANCA-associated glomerulonephritis/systemic vasculitis in childhood: clinical features–outcome. Pediatr Nephrol 27, 1911–1920 (2012). https://doi.org/10.1007/s00467-012-2198-5
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DOI: https://doi.org/10.1007/s00467-012-2198-5