Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)
Jazyk angličtina Země Velká Británie, Anglie Médium electronic-ecollection
Typ dokumentu srovnávací studie, časopisecké články, práce podpořená grantem
PubMed
24891844
PubMed Central
PMC4041043
DOI
10.1186/1546-0096-12-18
PII: 1546-0096-12-18
Knihovny.cz E-zdroje
- Klíčová slova
- Clinical picture of disease, Clinical study, Comparison with literature, Granulomatosis with polyangiitis, Wegener’s granulomatosis,
- MeSH
- biopsie MeSH
- dítě MeSH
- dospělí MeSH
- granulom dýchacího systému * imunologie patologie MeSH
- granulomatóza s polyangiitidou * diagnóza epidemiologie imunologie patofyziologie MeSH
- lidé MeSH
- mezinárodní spolupráce MeSH
- mladiství MeSH
- orgánová specificita imunologie MeSH
- předškolní dítě MeSH
- prognóza MeSH
- protilátky proti cytoplazmě neutrofilů krev MeSH
- věk při počátku nemoci MeSH
- věkové faktory MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- srovnávací studie MeSH
- Názvy látek
- protilátky proti cytoplazmě neutrofilů MeSH
BACKGROUND: Granulomatosis 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. FINDINGS: The 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. CONCLUSIONS: Paediatric patients compared to adults with GPA/WG have similar pattern of clinical manifestations but different frequencies of organ involvement.
Ann and Robert H Lurie Children's Hospital of Chicago Chicago IL USA
Asklepios Klinik Altona Hamburg Germany
Benghazi Children Hospital Benghazi MUB Rheumatology Clinic Benghazi Lybia
CHU Le Kremlin Bicêtre APHP University of Paris Sud CEREMAI Le Kremlin Bicêtre Paris France
Department of Pediatric Immunology and Rheumatology Wilhelmina Kinderziekenhuis Utrecht Netherlands
Department of Pediatric Rheumatology and Immunology University Children's Hospital Muenster Germany
Department of Pediatrics Emma Children Hospital AMC Amsterdam Netherlands
Department of Rheumatology Great Ormond St Hospital NHS Foundation Trust London UK
Dipartimento di Pediatria Università degli Studi di Genova Genova Italy
Hamburger Zentrum für Kinder und Jugendrheumatologie Klinikum Eilbek Hs 6 Hamburg Germany
Hospital Dr Felipe Glasman Rheumatolgy Section Bahía Blanca Buenos Aires Argentina
Istituto Giannina Gaslini Nefrologia Genova Italy
Istituto Giannina Gaslini Pediatria 2 Reumatologia PRINTO Genova Italy
Istituto Giannina Gaslini Servizio di Epidemiologia e Biostatistica Genova Italy
Juliane Marie Centret Rigshospitalet Pediatrisk klinik 2 Copenhagen Denmark
Pediatric Department University Hospital Centre Tirana Albania
Universidade Federal de São Paulo Pediatrics São Paulo Brazil
Cassidy JT, Petty RE, Laxer RM, Lindsley CB. Granulomatous vasculitis, giant cell arteritis and sarcoidosis. In: Textbook of pediatric rheumatology. Harcourt Health Science Company, editor. Sixth edition:315–343. Philadelphia, USA: W.B.Saunders Company; 2010.
