ORIGINAL ARTICLE ANCA-associated glomerulonephritis/systemic vasculitis in childhood: clinical features–outcome Ekaterini Siomou & Despoina Tramma & Claire Bowen & David V. Milford Received: 25 January 2012 / Revised: 21 April 2012 / Accepted: 1 May 2012 / Published online: 31 May 2012 # IPNA 2012 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 ne- phrology department. Results Thirteen cases of AAGNV were identified: seven Wegener granulomatosis (WG) and six microscopic poly- angiitis (MPA). Acute renal failure/nephrotic range protein- uria (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 glo- merular filtration rate, oliguria, NRP, crescentic NGN, glo- merular 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 methyl- prednisolone, cyclophosphamide and plasma exchange in- duced extra-renal remission/serological response and renal function improvement/stabilization. Markedly decreased GFR, oliguria, NRP, and chronic glomerular lesions at pre- sentation 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 E. Siomou : D. Tramma : D. V. Milford Department of Nephrology, Birmingham Children’ s Hospital NHS Foundation Trust, Steelhouse Lane, Birmingham B4 6NH, UK C. Bowen Department of Paediatric Pathology, Birmingham Children’ s Hospital NHS Foundation Trust, Steelhouse Lane, Birmingham B4 6NH, UK E. Siomou (*) University Hospital of Ioannina, Stavros Niarchos Avenue, 45 500, Ioannina, Greece e-mail: eksiomou@yahoo.gr Pediatr Nephrol (2012) 27:1911–1920 DOI 10.1007/s00467-012-2198-5