Clinical analysis of 20 children with ANCA-associated small vasculitis

CHEN Zhi; ZHANG Gui-ju; SHEN Ying

Abstract：Objective To investigate the characteristics of ANCA-associated small vasculitis in children and to improve its recognition. Methods Twenty children patients with ANCA-associated small vasculitis were studied retrospectively by clinical manifestations, serum examination, pathological data, treatment and prognosis, etc. Results Among 20 cases, 9 patients were involved in the respiratory tract, 19 patients were involved in the lung and number of kidney in-volved was 14. MPO and PR3 were positive in 10 and 8 cases respectively; 14 children received pathological examina-tion (9 cases of kidney, 2 cases of nasal mucosa, 2 cases of skin and 1 case of lung). Twelve cases were treated with corticosteroid and cyclophosphamide and 6 cases with corticosteroid. Treatment effectiveness was reliable. Clinical status improved in 10 cases, 8 children gave up treatment and were lost, and 2 cases died. Conclusion ANCA-associated small vasculitis in children has diverse clinical manifestations. The upper and lower respiratory tract and kidney are most commonly involved organs. ANCA inspection is characteristic. Corticosteroid and immunosuppressive treatment can achieve good outcome. It is difficult to diagnose this disease.