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Title: Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)
Authors: Bohm, Marek
Fernandez, Maria Isabel Gonzalez
Ozen, Seza
Pistorio, Angela
Dolezalova, Pavla
Brogan, Paul
Barbano, Giancarlo
Sengler, Claudia
Klein-Gitelman, Marisa
Quartier, Pierre
Fasth, Anders
Herlin, Troels
Terreri, Maria Teresa R. A. [UNIFESP]
Nielsen, Susan
van Rossum, Marion A. J.
Avcin, Tadej
Rodolfo Castell, Esteban
Foeldvari, Ivan
Foell, Dirk
Kondi, Anuela
Kone-Paut, Isabelle
Kuester, Rolf-Michael
Michels, Hartmut
Wulffraat, Nico
Ben Amer, Halima
Malattia, Clara
Martini, Alberto
Ruperto, Nicolino
Paediat Rheumatology Int
Charles Univ Prague
Gen Univ Hosp Prague
Hacettepe Univ
Ist Giannina Gaslini
NHS Fdn Trust
Ann & Robert H Lurie Childrens Hosp Chicago
Univ Paris 05
Univ Gothenburg
Aarhus Univ Hosp
Universidade Federal de São Paulo (UNIFESP)
Emma Children Hosp AMC
Univ Childrens Hosp
Hosp Dr Felipe Glasman
Klinikum Eilbek Hs 6
Univ Hosp Ctr
Univ Paris 11
Asklepios Klin Altona
Kinderklin Garmisch Partenkirchen gGmbH
Wilhelmina Childrens Hosp
Benghazi Children Hosp Benghazi
Univ Genoa
Keywords: Wegener's granulomatosis
Granulomatosis with polyangiitis
Clinical study
Clinical picture of disease
Comparison with literature
Issue Date: 26-May-2014
Publisher: Biomed Central Ltd
Citation: Pediatric Rheumatology. London: Biomed Central Ltd, v. 12, 5 p., 2014.
Abstract: 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.
ISSN: 1546-0096
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