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    Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)
    (Biomed Central Ltd, 2014-05-26) 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; PRINTO; Charles Univ Prague; Gen Univ Hosp Prague; Hacettepe Univ; Ist Giannina Gaslini; NHS Fdn Trust; Charite; Ann & Robert H Lurie Childrens Hosp Chicago; Univ Paris 05; Univ Gothenburg; Aarhus Univ Hosp; Universidade Federal de São Paulo (UNIFESP); Rigshosp; 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
    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.