ArticlePediatric rheumatology online journal2014
Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis).
Article in Pediatric rheumatology online journal, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers, 2 of them syntheses that pooled it.
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Who cites it
37 citing papers in PubMed, 2 syntheses or guidelines pooled it, 109 citations in OpenAlex.
- A systematic review exploring the bidirectional relationship between puberty and autoimmune rheumatic diseases.Pediatric rheumatology online journal · 2021Pooled it
- Childhood-onset granulomatosis with polyangiitis and microscopic polyangiitis: systematic review and meta-analysis.Orphanet journal of rare diseases · 2016Pooled it
- Autoantibodies Against Lysosome Associated Membrane Protein-2 (LAMP-2) in Pediatric Chronic Primary Systemic Vasculitis.Frontiers in immunology · 2020Trial
- Tracheobronchial Stenosis in Granulomatosis With Polyangiitis: Immunosuppressant Use and Airway Dilation Frequency.ACR open rheumatology · 2026Article
- Granulomatosis With Polyangiitis Presenting as Deep Vein Thrombosis in an Adolescent: A Rare Case Report.Clinical case reports · 2026Article
- An atypical case of granulomatosis with polyangiitis in a 15-year-old girl presenting with pyoderma gangrenosum-like ulcers on the face.Dermatology online journal · 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
- An unusual case of pediatric granulomatosis with polyangiitis complicated by splenic infarction presenting as inflammatory bowel disease.JPGN reports · 2024Article
- Article
- Rare central nervous system manifestation of granulomatosis with polyangiitis in a 12-year-old child: A case report.Radiology case reports · 2023Article
- Multiple Giant Coronary Artery Aneurysms in a Pediatric Patient with Granulomatosis with Polyangiitis.Pediatric cardiology · 2022Article
- Article
- Infliximab therapy of relapsing tracheal stenosis in a pediatric patient with granulomatosis with polyangiitis: a case report.Journal of medical case reports · 2022Article
- Article
- Granulomatosis with Polyangiitis in Adolescence: Two Distinct Presentations.Case reports in rheumatology · 2021Article
- Article
- Coughing up clues: 16-year-old girl with acute haemoptysis.BMJ case reports · 2020Article
- Giant Thrombosis at Left Anterior Descending Artery Aneurysm in a 10-Year Old Boy with Granulomatosis with Polyangiitis.Case reports in cardiology · 2020Article
Corrections and comments
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Authors and funding
28 authors at 20 institutions in 13 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundGranulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), belongs to the group of ANCA-associated necrotizing vasculitides. This study describes the clinical picture of the disease in a large cohort of GPA paediatric patients. Children with age at diagnosis ≤ 18 years, fulfilling the EULAR/PRINTO/PRES GPA/WG classification criteria were extracted from the PRINTO vasculitis database. The clinical signs/symptoms and laboratory features were analysed before or at the time of diagnosis and at least 3 months thereafter and compared with other paediatric and adult case series (>50 patients) derived from the literature.
findingsThe 56 children with GPA/WG were predominantly females (68%) and Caucasians (82%) with a median age at disease onset of 11.7 years, and a median delay in diagnosis of 4.2 months. The most frequent organ systems involved before/at the time of diagnosis were ears, nose, throat (91%), constitutional (malaise, fever, weight loss) (89%), respiratory (79%), mucosa and skin (64%), musculoskeletal (59%), and eye (35%), 67% were ANCA-PR3 positive, while haematuria/proteinuria was present in > 50% of the children. In adult series, the frequency of female involvement ranged from 29% to 50% with lower frequencies of constitutional (fever, weight loss), ears, nose, throat (oral/nasal ulceration, otitis/aural discharge), respiratory (tracheal/endobronchial stenosis/obstruction), laboratory involvement and higher frequency of conductive hearing loss than in this paediatric series.
conclusionsPaediatric patients compared to adults with GPA/WG have similar pattern of clinical manifestations but different frequencies of organ involvement.
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