title: ANCA-associated glomerulonephritis in systemic-onset juvenile idiopathic arthritis.
abstract: 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.
authors.affiliation: Service de Néphrologie et Rhumatologie Pédiatriques et CNRS UMR, Hôpital Femme Mère Enfant, Lyon, France. alexandre.belot@chu-lyon.fr
authors.forename: Alexandre
authors.initials: A
authors.lastname: Belot
authors.forename: Brigitte
authors.initials: B
authors.lastname: Bader-Meunier
authors.forename: Patrick
authors.initials: P
authors.lastname: Niaudet
authors.forename: Rémi
authors.initials: R
authors.lastname: Salomon
authors.forename: Anne-Marie
authors.initials: AM
authors.lastname: Prieur
authors.forename: Laure-Hélène
authors.initials: LH
authors.lastname: Noel
authors.forename: Pierre
authors.initials: P
authors.lastname: Quartier
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic; D001171:Arthritis, Juvenile / Q000150:complications*; D002675:Child, Preschool; D005260:Female; D005921:Glomerulonephritis / Q000150:complications* / Q000276:immunology; D006801:Humans; D007223:Infant; D008297:Male
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic