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

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. © 2014 bohm et al.; licensee biomed central ltd.