ArticlePediatric nephrology (Berlin, Germany)2023
Pediatric ANCA vasculitis: clinical presentation, treatment, and outcomes in a French retrospective study.
Article in Pediatric nephrology (Berlin, Germany), 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed, 13 citations in OpenAlex.
- Outcomes and Validation of Histopathological Scores in Pediatric ANCA-vasculitis.Kidney international reports · 2026Article
- Antineutrophil Cytoplasmic Antibodies (ANCAs): Role in Diagnosis, Disease Monitoring, and Prognosis.Journal of the American Society of Nephrology : JASN · 2025Review
- 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
- Renal disease in pediatric rheumatology.Current opinion in rheumatology · 2024Review
- Childhood-Onset ANCA- Associated Vasculitis: single center experience from Central California.Pediatric rheumatology online journal · 2023Article
Corrections and comments
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Authors and funding
21 authors at 14 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPediatric ANCA vasculitis is a rare group of diseases with a scarcity of data in children. Annual incidence appeared to increase in the last several years, placing higher interest in the clinical and therapeutical outcomes of the disorder. Also, the growing use of rituximab questions the latest outcomes in these diseases. We therefore conducted a retrospective study to better understand the current characteristics, management, and the latest outcomes of the disorder.
methodsWe conducted a 9-year retrospective study of 46 children in 14 different centers across France to describe their clinical and laboratory presentations, therapeutic regimens, and kidney outcome.
resultsP-ANCA appeared to be a potential marker for higher relapse risk. Compared to adults, we found that ear-nose-throat presentations were frequent (45.7%) and more severe. Despite an evolution in the treatment management, kidney outcome remained poor with a substantial proportion of chronic kidney disease (54.8% at 1 year). Mortality stays low with 3 patients (6.5%) deceased at the end of our study.
conclusionClinical presentation was as previously described and time to diagnosis remains long. P-ANCA is a statistically significant marker for increased relapse risk. We observed a modification in the treatment regimens over the past several years with a growing use of rituximab and a decreasing use of cyclophosphamide. Despite these changes, kidney outcome remains poor and prospective studies should be conducted to assess the most appropriate therapeutic modality for each patient. A higher resolution version of the Graphical abstract is available as Supplementary information.
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