Volume 9 Supplement 1
Clinical features of anti-neutrophil cytoplasmic antibody-associated systemic vasculitis in French children
© Sacri et al; licensee BioMed Central Ltd. 2011
Published: 14 September 2011
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is mainly reported in adults. Data in children is scarce. The current study aimed to describe the clinical features of the disease at diagnosis and the long-term outcomes in pediatric patients with ANCA-associated vasculitis.
This retrospective study was conducted in 9 French Hospitals (mainly pediatric nephrology units) and includes patients diagnosed during the past 25 years. Wegener’s granulomatosis (WG) and microscopic polyangiitis (MPA) were included, but not Churg-Strauss syndrome.
Forty-seven children were included, 15 (32%) with WG and 32 (68%) with MPA, 77% children were pANCA+/MPO. Female predominated (85%), the median age at diagnosis was 10 years (2-16), and average time to diagnosis was 8 months (0-58). 53% children presented with fever, 77% with deterioration of general condition. 91% had renal involvement. Among them 91% had proteinuria, 44% had hypertension, 79% had renal failure of whom one third presented with end-stage renal disease (ESRD), 22% had gross hematuria and 82% microscopic hematuria. Half the patients showed pulmonary involvement (alveolar hemorrhage for 77% and nodules for 18%). 28% presented with upper airways involvement, 38% with cutaneous lesions (67% purpura), and 19% with arthritis. Few had digestive, ophthalmic or CNS involvement. After an average follow-up of 5 years (2m-24y), 3 patients (6%) died. 60% were considered in remission (partial or total) after induction treatment and 77% at last follow-up. 33% of children with initial renal involvement developed chronic kidney disease, half of them progressed to ESRD. 43% needed dialysis and 29% underwent a renal transplantation.
Clinical features and outcome in children with ANCA-vasculitis are close to the findings in adults but they are characterized by delayed diagnosis and female predominance. Although the cases may be selected, renal involvement appears as a major prognostic factor of the disease.
This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.