儿童韦格纳肉芽肿10例临床分析

摘要

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.%目的 分析儿童韦格纳肉芽肿的特点,提高对该病的认识.方法 对10例韦格纳肉芽肿患儿的临床表现、辅助检查、病理结果、治疗等资料进行总结分析.结果 10例患儿中上呼吸道、肺脏受累10例,肾脏受累6例,关节、皮肤、眼及神经系统等也有不同程度受累.实验室检查胞浆型抗中性粒细胞胞浆抗体(cANCA)阳性8例;5例行病理检查(肾脏1例,鼻黏膜2例,皮肤2例),均表现血管炎和(或)肉芽肿性改变,肾活检有新月体形成.7例患儿给予糖皮质激素(甲基泼尼松龙冲击)联合环磷酰胺治疗,1例进行糖皮质激素联合氨甲蝶呤治疗,患儿临床表现均有不同程度的改善.结论 儿童韦格纳肉芽肿临床表现多样,误诊率高;主要累及呼吸道及肾脏,ANCA检查有特异性;糖皮质激素结合免疫抑制剂治疗有效.