ABSTRACT
Objective To investigate the characteristics of Wegener granulomatosis in children and to improve its recognition. Methods Ten pediatric patients with Wegener granulomatosis were studied retrospectively by clinical manifestations, serum examination, pathological data, treatment, etc. Results All children had clinical evidence of upper respiratory tract and lungs involvement; kidney lesion occurred in 6 cases(6/10); joints, skin, eyes and nervous system were also involved with different degree, cANCA (PR3) showed positive in 8 cases; Pathological examination (1 kidney sample, 2 nasal mucosa samples, 2 skin samples) showed granulomatous vasculitis and crescents were found in renal biopsy; 7 cases were treated with corticosteroid plus cyclophosphamide, 1 case was treated with corticosteroid plus methotrexate, and improvement were seen in all of them. Conclusions Wegener granulomatosis in children have diverse clinical manifestations and clinical diagnosis is difficult. The upper and lower respiratory tract and kidney are most commonly involved organs. ANCA inspection is characteristic. Treatment with corticosteroid and immunosuppressive can achieve good outcome.