SynthesisOrphanet journal of rare diseases2016
Childhood-onset granulomatosis with polyangiitis and microscopic polyangiitis: systematic review and meta-analysis.
Synthesis in Orphanet journal of rare diseases, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
38 citing papers in PubMed, 89 citations in OpenAlex.
- Therapeutic plasma exchange in paediatric ANCA-associated vasculitis: a multicentre real-world experience.Rheumatology (Oxford, England) · 2026Observational
- From ANCA-mediated vascular injury to coronary microvascular and myocardial involvement in granulomatosis with polyangiitis: an immunocardiology perspective.Rheumatology international · 2026Review
- Immune-mediated cochleovestibular dysfunction: clinical spectrum from isolated inner-ear disorders to systemic autoimmune diseases and therapeutic strategies.Frontiers in immunology · 2026Review
- Delayed Diagnosis of Pulmonary-Renal Syndrome Presenting as Chronic Hemoptysis Leading to ESRD.Case reports in pediatrics · 2026Article
- Infertility as a Rare Manifestation of Pituitary Involvement in Granulomatosis With Polyangiitis: A Case Report.Cureus · 2025Article
- In-Depth Analysis of Disease Manifestations in Antineutrophil Cytoplasmic Antibody-Associated Vasculitides Identifies Distinct Clinical Phenotypes.ACR open rheumatology · 2025Article
- Respiratory manifestations of pediatric granulomatosis with polyangiitis: A 12-year experience from a tertiary care facility.Pediatric pulmonology · 2025Article
- Pediatric Presentations of Granulomatosis With Polyangiitis: A Double Case Study.Case reports in dermatological medicine · 2025Article
- Childhood-Onset ANCA-Associated Vasculitis: From Genetic Studies to Advances in Pathogenesis, Classification and Novel Therapeutic Approaches.International journal of molecular sciences · 2024Review
- Vasculitis in Children.Turkish archives of pediatrics · 2024Review
- [Clinical characteristics of children with anti-neutrophil cytoplasmic antibody-associated vasculitis].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2024Article
- The Diagnostic Role of Skin Manifestations in Rheumatic Diseases in Children: A Critical Review of Paediatric Vasculitis.International journal of molecular sciences · 2024Review
- The Role of Neutrophils in ANCA-Associated Vasculitis: The Pathogenic Role and Diagnostic Utility of Autoantibodies.International journal of molecular sciences · 2023Review
- Pediatric ANCA vasculitis: clinical presentation, treatment, and outcomes in a French retrospective study.Pediatric nephrology (Berlin, Germany) · 2023Article
- Article
- Granulomatosis with Polyangiitis Misdiagnosed as IgA Vasculitis in a Child.Case reports in pediatrics · 2023Article
- Diffuse alveolar haemorrhage in children hospitalised in a tertiary‑level hospital: A retrospective descriptive study.African journal of thoracic and critical care medicine · 2023Article
- Pulmonary renal syndrome: a clinical review.Breathe (Sheffield, England) · 2022Review
- Granulomatosis with polyangiitis in an adolescent male with chronic cough and pulmonary nodules.BMJ case reports · 2022Article
- Systematic Review and Metaanalysis of Worldwide Incidence and Prevalence of Antineutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis.Journal of clinical medicine · 2022Article
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThe data from cohorts of childhood-onset granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) remain scarce and heterogeneous. We aimed to analyse the features at presentation, therapeutic approaches and the disease course of these rare diseases.
methodsElectronic searches of Medline and the Cochrane Central Register of Controlled trials database were conducted. We also checked the reference lists of the studies included and other systematic reviews, to identify additional reports. We included all cohorts, cross-sectional studies or registries reporting features at presentation or outcomes in patients with a diagnosis of childhood-onset GPA or MPA (age <18 years). The pooled prevalence of clinical manifestations at presentation, ANCA and induction therapies for GPA and MPA was calculated.
resultsWe reviewed 570 full texts and identified 14 studies on GPA and 8 on MPA. Childhood-onset GPA and MPA occurred predominantly in female subjects during adolescence. For GPA, ear-nose-throat (ENT) disease (pooled prevalence 82 % [95 % CI 78-87]), constitutional symptoms (73 % [95 % CI 55-88]), renal (65 % [95 % CI 49-79]), and lower respiratory tract (61 % [95 % CI 48-74]) manifestations were the most frequently reported at presentation. Renal disease was a hallmark of MPA (94 % [95 % CI 89-97]). ANCA were detected in >90 % of children with GPA or MPA. Combined corticosteroids and cyclophosphamide was the most frequently used first remission-inducing treatment for GPA (76 % [95 % CI 69-82]) and MPA (62 % [95 % CI 20-96]). Relapses occurred more frequently in GPA (67-100 %) than in MPA (25-50 %). The leading causes of death were the disease itself, and infections.
conclusionsChildhood-onset MPA and GPA remain severe diseases with frequent relapses and a high cumulative morbidity. Survival and disease-free survival need to be improved.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.