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Belot, Alexandre, MD; Bader-Meunier, Brigitte, MD; Niaudet, Patrick, MD; Salomon, Rémi, MD, PhD; Prieur, Anne-Marie, MD; Noel, Laure-Hélène, MD; Quartier, Pierre, MD
American journal of kidney diseases, 03/2012, Volume: 59, Issue: 3Journal Article
Systemic-onset juvenile idiopathic arthritis is an inflammatory disease of unknown cause and is not commonly associated with kidney involvement. We describe 3 patients with systemic-onset juvenile idiopathic arthritis with high disease activity who developed antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis 1-6 years after the onset of systemic-onset juvenile idiopathic arthritis. Renal and systemic-onset juvenile idiopathic arthritis remission occurred in one patient under anti–interleukin 1 (anti–IL-1) treatment associated with immunosuppressive drugs. The other 2 patients developed end-stage renal disease, and one of those patients died. This report suggests that the diagnosis of ANCA-associated glomerulonephritis must be considered in patients with systemic-onset juvenile idiopathic arthritis with persistently active systemic disease who present with proteinuria. Furthermore, use of an anti–IL-1 agent might be an effective therapeutic option.
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