儿童抗中性粒细胞胞浆抗体相关小血管炎20例临床分析

Clinical analysis of 20 children with ANCA-associated small vasculitis

目的分析儿童抗中性粒细胞胞浆抗体相关小血管炎(AASV)的特点,提高对本病的认识。方法2001年6月至2008年11月首都医科大学附属北京儿童医院共诊断儿童原发ANCA相关小血管炎患儿20例,对其临床表现、辅助检查、病理结果、治疗及预后等资料进行总结分析。结果20例患儿中,呼吸道受累9例、肺脏受累19例、肾脏受累14例;实验室检查髓过氧化物酶(MPO)阳性10例、蛋白酶3(PR3)阳性7例;14例行病理检查,其中肾脏病理检查9例、鼻黏膜病检2例、皮肤活检2例、肺活检1例。20例患儿中18例给予糖皮质激素治疗(其中12例接受糖皮质激素联合免疫抑制剂治疗),2例未接受激素或免疫抑制剂治疗。最终10例患儿好转出院(其中4例继续间断返院行甲泼尼龙联合环磷酰胺冲击治疗),8例患儿因经济等原因放弃继续治疗并失访,2例死亡。结论儿童AASV临床表现多样,主要累及上、下呼吸道及肾脏,ANCA化验有特异性,糖皮质激素联合免疫抑制剂治疗有效。本病临床诊断率低、误诊率高。

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.