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

Bohm, M.; Gonzalez Fernandez, M. I.; Ozen, S.; Pistorio, A.; Dolezalova, P.; Brogan, P.; Barbano, G.; Sengler, C.; Klein-Gitelman, M.; Quartier, P.; Fasth, A.; Herlin, T.; Terreri, M. T.; Nielsen, S.; van Rossum, M. A.; Avcin, T.; Castell, E. R.; Foeldvari, I.; Foell, D.; Kondi, A.; Kone-Paut, I.; Kuester, R. M.; Michels, H.; Wulffraat, N.; Amer, H. B.; Malattia, C.; Martini, A.; Ruperto, N.

Pediatr Rheumatol Online J. 2014 Jun 4; 12:18


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 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.

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