TY - JOUR
T1 - Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)
AU - Bohm, Marek
AU - Gonzalez Fernandez, Maria Isabel
AU - Ozen, Seza
AU - Pistorio, Angela
AU - Dolezalova, Pavla
AU - Brogan, Paul
AU - Barbano, Giancarlo
AU - Sengler, Claudia
AU - Klein-Gitelman, Marisa
AU - Quartier, Pierre
AU - Fasth, Anders
AU - Herlin, Troels
AU - Terreri, Maria Teresa R. A.
AU - Nielsen, Susan
AU - van Rossum, Marion A. J.
AU - Avcin, Tadej
AU - Castell, Esteban Rodolfo
AU - Foeldvari, Ivan
AU - Foell, Dirk
AU - Kondi, Anuela
AU - Koné-Paut, Isabelle
AU - Kuester, Rolf-Michael
AU - Michels, Hartmut
AU - Wulffraat, Nico
AU - Amer, Halima Ben
AU - Malattia, Clara
AU - Martini, Alberto
AU - Ruperto, Nicolino
PY - 2014
Y1 - 2014
N2 - 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
AB - 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
U2 - https://doi.org/10.1186/1546-0096-12-18
DO - https://doi.org/10.1186/1546-0096-12-18
M3 - Article
C2 - 24891844
SN - 1546-0096
VL - 12
SP - 18
JO - Pediatric rheumatology online journal
JF - Pediatric rheumatology online journal
ER -