Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)

• Autor(a) principal: Bohm, Marek
• Data de Publicação: 2014
• Outros Autores: 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
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Institucional da UNIFESP
• Texto Completo: http://repositorio.unifesp.br/handle/11600/37782; http://dx.doi.org/10.1186/1546-0096-12-18
• topic: Wegener's granulomatosis; Granulomatosis with polyangiitis; Clinical study; Clinical picture of disease; Comparison with literature

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.