ArticlePediatric nephrology (Berlin, Germany)2019
Epidemiology and clinical features of childhood-onset anti-neutrophil cytoplasmic antibody-associated vasculitis: a clinicopathological analysis.
Article in Pediatric nephrology (Berlin, Germany), 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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14 citing papers in PubMed, 25 citations in OpenAlex.
- Pediatric case of anti-neutrophil cytoplasmic antibody-associated glomerulonephritis with immunoglobulin a deposition diagnosed early through school urinary screening.CEN case reports · 2026Article
- Microscopic Polyangiitis Following Kawasaki Disease in a Patient With Autoimmune Predisposition.Cureus · 2026Article
- Single-Cell RNA-Sequencing of Peripheral Blood Reveals Neutrophil Heterogeneity in Childhood ANCA-Associated Vasculitis.Journal of inflammation research · 2026Article
- Expert consensus on the off-label use of drugs for pediatric rare diseases in China (2025 edition).Translational pediatrics · 2025Review
- New avenues in childhood vasculitis.Pediatric rheumatology online journal · 2025Review
- Antineutrophil Cytoplasmic Antibodies (ANCAs): Role in Diagnosis, Disease Monitoring, and Prognosis.Journal of the American Society of Nephrology : JASN · 2025Review
- Performance of EMA algorithm, 2022 ACR/EULAR criteria, and EMA-ACR/EULAR algorithm in classifying pediatric ANCA-associated vasculitis: a national cohort study in China.World journal of pediatrics : WJP · 2025Article
- Respiratory manifestations of pediatric granulomatosis with polyangiitis: A 12-year experience from a tertiary care facility.Pediatric pulmonology · 2025Article
- Childhood-Onset ANCA-Associated Vasculitis: From Genetic Studies to Advances in Pathogenesis, Classification and Novel Therapeutic Approaches.International journal of molecular sciences · 2024Review
- Clinicopathological characteristics of pediatric ANCA-associated glomerulonephritis.Pediatric nephrology (Berlin, Germany) · 2024Article
- Risk factors for renal outcomes in children with antineutrophil cytoplasmic antibody-associated vasculitis: a nationwide retrospective study in China.World journal of pediatrics : WJP · 2024Article
- Renal pathology in adult and paediatric population of Japan: review of the Japan renal biopsy registry database from 2007 to 2017.Journal of nephrology · 2023Review
- Atypical Neurological Manifestation in Childhood Microscopic Polyangiitis: A Case Report and Review of Literature.Frontiers in pediatrics · 2022Article
- Recent Advances in Pediatric Vasculitis.Rheumatic diseases clinics of North America · 2021Review
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Authors and funding
7 authors at 6 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThis study was performed to determine the clinical features and outcomes of childhood-onset anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA).
methodsA retrospective Japanese multicenter study was performed in patients diagnosed with AAV before 16 years of age.
resultsOf 49 patients with AAV, 36 were female. The diagnoses were as follows: MPA (n = 38, 78%), granulomatosis with polyangiitis (GPA; n = 9, 18%), eosinophilic granulomatosis with polyangiitis (EGPA; n = 1, 2%), and other (n = 1, 2%). The median age at onset was 10.7 years, and median time to diagnosis was 2.0 months. Twenty-seven (55%) patients were identified through a school urinary screening program. Initial symptoms included fever and fatigue (45%), and renal (71%), pulmonary (29%), ocular (20%), and mucocutaneous involvement (22%). Although 27 (55%) patients achieved remission and none had died at the last follow-up, at least one recurrence occurred in 13 (48%) patients after a median of 48 months and was more common in patients with GPA (P < 0.01). After a median follow-up of 43 months, seven (14%) patients (all with MPA) progressed to end-stage renal disease (ESRD).
conclusionsChildhood-onset AAV has an estimated prevalence of 3.41-4.28 per million children and is characterized by female predominance and high frequency of detection in school urinary screening programs. More than 10% of patients with childhood-onset AAV still progress to ESRD without achieving remission. Histological chronicity is a factor associated with ESRD.
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