ArticlePediatric nephrology (Berlin, Germany)2012
ANCA-associated glomerulonephritis/systemic vasculitis in childhood: clinical features-outcome.
Article in Pediatric nephrology (Berlin, Germany), 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 48 citations in OpenAlex.
- Granulomatosis with Polyangiitis: A Focus on Differences and Similarities Between Child and Adult Patients.Medicina (Kaunas, Lithuania) · 2025Review
- Pediatric ANCA vasculitis: clinical presentation, treatment, and outcomes in a French retrospective study.Pediatric nephrology (Berlin, Germany) · 2023Article
- Clinicopathological Analysis of 34 Cases of Primary Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in Chinese Children.Frontiers in pediatrics · 2021Article
- Age-dependent survival in rapidly progressive glomerulonephritis: A nationwide questionnaire survey from children to the elderly.PloS one · 2020Article
- Epidemiology and clinical features of childhood-onset anti-neutrophil cytoplasmic antibody-associated vasculitis: a clinicopathological analysis.Pediatric nephrology (Berlin, Germany) · 2019Article
- Genetics of COPA syndrome.The application of clinical genetics · 2019Review
- Adolescent PR3-ANCA-positive hypertrophic pachymeningitis: A case report and review of the literature.Medicine · 2018Article
- Pathogenesis and treatment of ANCA-associated vasculitis-a role for complement.Pediatric nephrology (Berlin, Germany) · 2018Review
- Paediatric anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis: an update on renal management.Pediatric nephrology (Berlin, Germany) · 2018Review
- Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis.Pediatric nephrology (Berlin, Germany) · 2017Article
- Unilateral Periorbital Swelling in Two Previously Healthy Females.Global pediatric health · 2017Article
- Copa Syndrome: a Novel Autosomal Dominant Immune Dysregulatory Disease.Journal of clinical immunology · 2016Review
- Pauci-immune glomerulonephritis in children: a clinicopathologic study of 21 patients.Pediatric nephrology (Berlin, Germany) · 2015Article
- [Atypical presentation of granulomatosis with polyangiitis: about a pediatric observation].The Pan African medical journal · 2015Article
- Clinicopathological characteristics and outcomes of pediatric patients with systemic small blood vessel vasculitis.Pediatric nephrology (Berlin, Germany) · 2014Article
- Tandem hemodialysis and plasma exchange.Pediatric nephrology (Berlin, Germany) · 2014Review
- Granulomatosis with polyangiitis: rapidly progressive necrotizing glomerulonephritis in a pediatric patient.International journal of nephrology and renovascular disease · 2014Article
- Microscopic polyangiitis complicated by intracerebral hemorrhage and pulmonary hemorrhage in a pediatric patient.The American journal of case reports · 2013Article
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAntineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis and systemic vasculitis (AAGNV) is uncommon in childhood.
methodsThis is a retrospective study of AAGNV cases diagnosed over a 13-year period in a tertiary pediatric nephrology department.
resultsThirteen cases of AAGNV were identified: seven Wegener granulomatosis (WG) and six microscopic polyangiitis (MPA). Acute renal failure/nephrotic range proteinuria (NRP) was found in 77 % of the patients (4 with WG, all with MPA). Eleven (85 %) patients showed necrotizing glomerulonephritis (NGN), with ≥50 % crescents identified in nine patients (69 %) (4 with WG, 5 with MPA). Treatment with methylprednisolone, cyclophosphamide and plasma exchange resulted in extra-renal remission and antibody reduction in all patients and renal function improvement/stabilization in 77 % of the patients. Three patients, all without oliguria at presentation and few sclerotic lesions, had normal renal function at follow-up. Chronic kidney disease (CKD) stages 2 and 3-4 were observed in four (WG) and three (MPA) patients, respectively. Three patients (23 %) developed end stage renal disease: two were MPA patients with severe presentation (markedly impaired glomerular filtration rate, oliguria, NRP, crescentic NGN, glomerular sclerosis) and one was a WG patient with extensive interstitial fibrosis/tubular atrophy.
conclusionsSevere renal involvement was more common in children with MPA than WG. Treatment with methylprednisolone, cyclophosphamide and plasma exchange induced extra-renal remission/serological response and renal function improvement/stabilization. Markedly decreased GFR, oliguria, NRP, and chronic glomerular lesions at presentation were predictors of poor outcome.
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