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中国循证儿科杂志

中国循证儿科杂志 ›› 2012, Vol. 7 ›› Issue (1): 59-62.

• 论著 • 上一篇    下一篇

儿童韦格纳肉芽肿病10例临床分析

马明圣,宋红梅,何艳燕,魏珉   

  1. 中国医学科学院 北京协和医学院 北京协和医院儿科
  • 收稿日期:2011-11-03 修回日期:2012-01-04 出版日期:2012-01-10 发布日期:2012-03-15
  • 通讯作者: 宋红梅

Clinical analysis of 10 children with Wegener’s granulomatosis

  • Received:2011-11-03 Revised:2012-01-04 Online:2012-01-10 Published:2012-03-15

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摘要： 目的 探讨韦格纳肉芽肿病(WG)的临床特征和预后。 方法 回顾性收集1990年10月至2010年7月在北京协和医院儿科确诊为WG、年龄<18岁且随访时间>3个月的患儿，提取临床表现、实验室检查、影像学检查、病理学检查和随访等资料进行分析。 结果 10例确诊WG患儿进入分析，其中男6例，女4例。发病年龄7~17.1岁，中位年龄13.9岁。从发病至确诊WG的病程为2~24个月。随访时间4个月至19年。①起病时发热7例，乏力3例，体重下降2例。病程中上呼吸道、肺脏和肾脏受累分别为10、8和4例，皮肤和眼部受累各3例，关节、消化系统和神经系统受累各2例。②所有患儿均行抗中性粒细胞胞质抗体(ANCA)检查，8例c-ANCA阳性，其中1例c-ANCA和p-ANCA均为阳性。③9例行鼻窦X线或CT检查,其中表现为鼻窦炎4例，鼻窦占位3例，侵犯眶内2例。8例行胸部CT检查，表现为肺内多发结节伴或不伴空洞形成5例，浸润性病灶2例，胸腔积液1例。2例行头颅MRI检查，均提示有异常信号，为脑缺血性改变。④10例患儿均行病理学检查，其中鼻黏膜活检7例，肺部活检2例，肾脏活检1例，病理改变主要为坏死性肉芽肿和（或）血管炎。⑤10例患儿均给予糖皮质激素联合环磷酰胺诱导缓解治疗，治疗后均达到临床缓解，维持治疗除给予糖皮质激素外分别加用环磷酰胺、甲氨蝶呤或环孢素等治疗。⑥随访期间7例患儿出现病情复发。1例患儿治疗17个月后因合并肺部感染和消化道出血死亡。1例出现了肾功能不全，需长期透析治疗。 结论 儿童WG临床表现多样，主要累及上呼吸道、肺脏和肾脏，c-ANCA阳性有助于诊断，组织活检可提供病理学诊断依据。糖皮质激素联合免疫抑制剂治疗可取得较好的疗效。

关键词: Children, Clinical feature, Prognosis, Wegener′s granulomatosis, 儿童, 临床表现, 韦格纳肉芽肿病, 预后

Abstract: Objective To describe the clinical features and outcome of Wegener′s granulomatosis in children. Methods A retrospective study of 10 patients diagnosed as Wegener′s granulomatosis was conducted at Department of Pediatrics, Peking Union Medical College Hospital from October 1990 to July 2010 to analyze the clinical manifestations, examination results, pathological data, treatment and outcome. Results Of 10 patients, 6 were males and 4 were females. The ages of patients at disease onset varied from 7 to 17.1 years. The median age was 13.9 years. Time for diagnosis ranged from 2 to 24 months and follow-up time ranged from 4 months to 19 years. ①Seven children presented with fever at disease onset, fatigue in 3 patients and weight loss in 2 patients. Upper airway involvement occurred in all patients. Eight patients had pulmonary involvement. Renal involvement occurred in 4 patients. Joints, skin, eyes and nervous system were rarely involved. ②c-ANCA positive reaction was seen in 8 patients. ③Sinus imaging studies were taken in 9 patients, sinusitis in 4 patients, sinus mass in 3 patients and violation of eyes in 2 patients. Eight patients underwent chest CT examination, multiple pulmonary nodules with or without cavity formation in 5 patients, invasive lesions in 2 patients, and pleural effusion in 1 patient. Brain MRI showed ischemic changes in 2 patients. ④Pathological examination (7 nasal mucosa samples, 2 lung samples, 1 kidney sample) showed necrotizing granuloma and (or) vasculitis. ⑤All children were given corticosteroids and cyclophosphamide at the beginning of treatment. Treatment was changed to use glucocorticoid and methotrexate in 2 patients and 1 patient was given cyclosporine A. All patients were remitted after treatment. ⑥Seven patients relapsed, 1 patient had renal insufficiency and 1 patient died of respiratory failure during the period of follow-up. Conclusions The upper respiratory tract, lung and kidney were the most commonly involved organs in Wegener′s granulomatosis. Positive c-ANCA and pathological examination were helpful to the diagnosis. Treatment with corticosteroid and immunosuppressive could achieve good outcome.