儿童抗中性粒细胞胞质抗体相关性血管炎患儿呼吸系统病变临床特征  

Respiratory features of children with antineutrophil cytoplasmic antibody - associated vasculitis

作　　者：张聪聪[1] 张明智[1] 孙利[2] 黄剑峰[1] 陆爱珍[1] 陆泳[1] 奚志敏[1] 王立波[1] 

机构地区：[1]复旦大学附属儿科医院呼吸科,上海201102 [2]复旦大学附属儿科医院风湿科,上海201102

出　　处：《中华实用儿科临床杂志》2016年第21期1627-1629,共3页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的探讨儿童抗中性粒细胞胞质抗体相关性血管炎（AAV）呼吸系统受累的临床表现、实验室及影像学特点。方法回顾性分析复旦大学附属儿科医院2005年12月至2016年2月诊治的12例AAV患儿的门诊和临床住院资料，对收集的资料进行描述性分析。结果12例患儿中男3例，女9例；平均年龄10.35岁。11例为显微镜下多血管炎（MPA），其中2例为药物继发性血管炎，分别为丙基硫氧嘧啶（PTU）和甲巯咪唑（MMI）；1例为肉芽肿性多血管炎（GPA）。所有病例确诊时间1～48个月，平均8个月。10例确诊时出现肺部受累，余2例随访1～62个月出现呼吸系统受累表现。确诊时表现为咳嗽8例，咳痰4例，咯血4例，呼吸困难4例，胸闷、胸痛2例，流涕、鼻塞2例，咽痛1例，喘息1例。均存在贫血及肾脏受累，发热10例，头晕乏力5例。影像表现多种多样，斑片状、团片状、条片状渗出影8例，磨玻璃影4例，囊状透亮影3例，结节影2例，条索状影2例，细小网格状影2例，细颗粒影1例。肺功能检查有小呼吸道功能下降、限制性病变及混合性病变等。结论对于不明原因贫血和肺部病变抗感染治疗不佳的学龄期女童，需考虑AAV的可能。AAV临床表现多样，缺乏特异性，极易误诊和延迟诊断。Objective To analyze the clinical,laboratory and radiological features of children with antineutro- phil cytoplasmic antibody - associated vasculitis （AAV） of the respiratory system. Methods Twelve children with AAV who were hospitalized from December 2005 to February 2016 in Children＇s Hospital of Fudan University, and de- scriptive analyzed outpatient and hospitalized clinical data were retrospectively analyzed. Results Of the 12 children, 9 cases were girls and 3 cases were boys with a mean age of 10.35 years old. Eleven children were categorized as micro- scopic polyangitis （ MPA）, including 2 cases of drug secondary vasculitis, propylthiouracil and methimazole respective- ly ; and 1 case was granulomatosis with polyangiitis （GPA）. The time of diagnosis varied from 1 to 48 months, mean time 8 months. Respiratory system involvement were present in 10 cases at diagnosis, and respiratory signs in the left 2 cases during the follow - up of 1 - 62 months. Symptoms included cough （ 8 cases）, sputum （4 cases）, hemoptysis （4 ca- ses） ,dyspnea （4 cases） ,chest pain （2 cases）,nasal obstruction and discharge （2 cases）,sore throat （1 case） and hyperpnea （ 1 case）. Other symptoms included anemia （ 12 cases ） , renal involvement （ 12 cases ）, fever （ 10 cases ）, dizziness （5 cases）. Imaging manifestations were different： exudation patchy and mass （ 8 cases）, ground - glass opacity （ 4 cases）, cystic lucency shadow （ 3 cases）, nodules （ 2 cases）, funicular shadow （2 cases）, fine grid opacities （2 ca- ses） and fine particles （1 case）. Pulmonary function tests showed a reduced small airway function, restricted lesions and mixed lesions, et al. Conclusions The clinical manifestations of AAV were varied and lack of specificity. More attention should be paid to AAV among school age girls with anemia and pulmonary diseases without response to antimi- crobial therapy.

关 键 词：抗中性粒细胞胞质抗体相关性血管炎 呼吸系统 儿童 

分 类 号：R725.9[医药卫生—儿科]