Abstract: 【Abstract】Objective To observe the effect of plasma exchange on anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis in children. Methods A total of 13 patients with ANCA-associated vasculitis treated in Hunan Children's Hospital from January 2017 to January 2020 were enrolled in this study, They were divided into two groups: control group (n=7), treated with glucocorticoid and immunosuppressive therapy; observation group (n=6), treated with plasma exchange other than the treatment in control group. Birmingham vasculitis activity score (BVAS), high resolution CT (HRCT) score, rate of negative ANCA, and renal
function were compared between the two groups. Results After the treatment,BVAS and HRCTscore were significantly lower than those before treatment in the two groups (For BVAS: 19.71±4.33 vs. 11.57±2.64, t=4.247, P=0.001 in control group; 20.50 ± 5.85 vs. 7.17 ± 2.56, t=3.552, P=0.005 in observation group. For HRCT score: 4.57±1.40 vs. 2.57±1.51, t=2.384, P=0.034 in control group; 4.17±1.17 vs. 0.83±0.65, t=5.583, P<0.001 in observation group). The decreases of BVAS and HRCT score were more in observation group than in control group (For BVAS: 7.17 ± 2.56 vs. 11.57 ± 2.64, t=3.037, P=0.011; for HRCT score: 0.83 ± 0.65 vs. 2.57 ± 1.51, t=2.413, P=0.034). The rate of negative ANCA was significantly higher in observation group than in control group (P=0.007). No significant improvement of renal function was found in both groups [Glomerular filtration rate before and after the treatment: 76.30(129.90, 30.59) vs. 98.88(141.89, 43.10)ml/(min·1.73m2), Z=0.943, P=0.346 for control group; 19.99(126.40, 15.02) vs. 17.80(145.19, 12.45) ml/(min·1.73m2), Z=0.689, P=0.491 for observation group]. Conclusion Plasma exchange combined with glucocorticoid and immunosuppressive therapy, can efficiently clear ANCA, reduce the activity of vasculitis and promote the recovery of lung lesions in children with ANCA-associated vasculitis.

Key words: Anti-neutrophil cytoplasmic autoantibody-associated vasculitis, Plasma exchange, Child