儿童韦格纳肉芽肿的临床和病理分析

目的 探讨儿童韦格纳肉芽肿(WG)的临床和病理特点.方法 收集本院2例和全国其他7例WG患儿,对其临床表现、实验室检查、影像学特点以及治疗进行分析.结果 9例患儿中男6例,女3例,平均年龄12岁,从发病到确诊的平均时间为8.3个月.有多系统、器官受累,以肾脏和肺最为常见.7例患儿进行抗中性粒细胞胞浆抗体(ANCA)检查,c-ANCA和抗PR3抗体阳性6例.8例患儿进行了组织活检,病理表现有实质组织坏死、肉芽肿和血管炎等.多数患儿接受糖皮质激素和环磷酰胺治疗后病情明显改善.结论 WG可侵犯多个组织器官,临床表现缺乏特异性,ANCA检查是早期诊断的关键.

Clinical and pathological analysis of Wegener granulomatosis in children

Objective To analyze the clinical and pathological features of Wegener granulomatosis (WG) in children in order to improve the understanding of this disease. Methods Nine cases of WG,including two cases from our hospital and seven cases throughout the country,were analyzed in aspects of their clinical characteristics,laboratory examinations,X-ray and treatments. Results Of the nine cases,six cases were male and three cases were female with average age of twelve years old. The average time from the initiation of clinical symptom to diagnosis was 8.3 months. Patients had multiple systems and organs involvements. Lung and kidney involvement were the most common. Six out of seven were positive in c-ANCA and anti-PR3 antibody. Biopsy was conducted in eight patients,and pathological manifestations involved parenchymal necrosis,granulomatosis,vasculitis and so on. Most patients improved significantly after being treated with glucocorticoids and cyclophosphamide. Conclusion WG is a syndrome which involves many organs without distinctive clinical manifestation. An ANCA test should be performed to make an early diagnosis.