摘要
目的探讨幼年特发性关节炎(JIA)并肺损伤患儿的临床特点、影像学表现及预后。方法回顾2016年1月至2019年9月于北京儿童医院连续收治的464例JIA患儿,分析其中合并肺损伤患儿的临床表现、肺部高分辨CT、肺功能及其临床随访情况。结果464例JIA患儿中合并肺损伤者40例(8.62%)。JIA合并肺损伤与未合并损伤患儿年龄、性别差异均无统计学意义(均P>0.05)。其中,全身型JIA(SoJIA)125例,合并肺损伤患儿28例(22.4%),在JIA合并肺损伤患儿中占比最高(70%,28/40例),40例患儿中出现呼吸道症状者22例(55.0%),明显缺氧表现者7例(17.5%);40例患儿全部进行肺部高分辨CT检查,影像表现为肺野内高密度条片或条絮影(75.0%,30/40例)、胸膜增厚(45.0%,18/40例)、磨玻璃影(22.5%,9/40例),小结节病灶(20.0%,8/40例)、小泡样或囊性气肿(15%,6/40例);肺功能检测20例患儿中12例出现不同程度肺功能异常表现,多表现为混合型通气功能障碍,其中2例治疗后仍遗留肺功能异常;随访3个月~3.5年,JIA合并肺损伤患儿死亡4例(10%),29例患儿治疗后复查肺CT,其中好转14例(48.28%),无明显好转者8例(27.58%),病情反复7例(24.14%)。结论JIA为儿童时期较为常见的风湿免疫系统疾病,其各分型均可并肺损伤,以肺间质病变为主,需引起临床重视。患儿年龄、性别无特异性。SoJIA出现肺损伤比例较其他各型更高,并巨噬细胞活化综合征(MAS)可导致呼吸衰竭,呼吸窘迫综合征,甚至死亡,是引起JIA预后不良的主要因素之一。肺部高分辨CT对于肺损伤的诊断更为敏感,肺功能检测简便易行,是评价、监测肺损伤的重要方式。JIA并肺损伤患儿预后较差,应引起临床关注。
Objective To explore clinical manifestations,imaging features and prognosis of juvenile idiopathic arthritis(JIA)combined with lung injury,aiming to improve the understanding of the disease.Methods Clinical data from 464 children with JIA who were hospitalized in Beijing Children′s Hospital from January 2016 to September 2019 were retrospectively analyzed.Their clinical manifestations,high resolution CT(HRCT)features,lung function and follow-up of children with lung injury were analyzed.Results Among 464 children with JIA,40 cases(8.62%)combined with lung injury.There were no significant differences in the age and sex between JIA children either combined with lung injury or not(all P>0.05).Among them,there were 125 cases of systemic-onset juvenile idiopathic arthritis(SoJIA)and 28 cases(22.4%)of JIA combined with lung injury,accounting for the highest proportion(70%,28/40 cases)in JIA children combined with lung injury.Among 40 JIA children combined with lung injury,22 cases(55.0%)had respiratory symptoms and 7 cases(17.5%)had obvious hypoxia.HRCT examination was performed in them,and the imaging findings included high-density strip or strip flocculation(75.0%,30/40 cases),pleural thickening(45.0%,18/40 cases),ground glass shadow(22.5%,9/40 cases),nodular lesion(20.0%,8/40 cases),vesicles or cystic emphysema(15.0%,6/40 cases).Lung function was detected in 12/20 children with varying degrees of pulmonary function abnormalities,most of which were mixed ventilation dysfunction,and 2 cases still had pulmonary function abnormalities after treatment.During the follow-up for 3 months to 3.5 years,4 cases(10%)JIA combined with lung injury died.A total of 29 children were re-examined by pulmonary CT in the follow-up visit,including 14(48.28%)improved,8 cases(27.58%)with no significant improvement,and 7 cases(24.14%)with repeated disease.Conclusions JIA is a common rheumatic immune disease in children and all subtypes can be combined with lung injury,manifesting as interstitial lung disease mainly.The age and sex of JIA
作者
袁昕
甄小芳
陈芳
胡艳
何强
郝静
莫鑫
Yuan Xin;Zhen Xiaofang;Chen Fang;Hu Yan;He Qiang;Hao Jing;Mo Xin(Department of Internal Medicine Comprehensive Ward 1,Beijing Children′s Hospital Affiliated to Capital Medical University,National Center for Children′s Health,Beijing 100045,China)
出处
《中华实用儿科临床杂志》
CAS
CSCD
北大核心
2021年第21期1605-1609,共5页
Chinese Journal of Applied Clinical Pediatrics
基金
中医药行业发展提质增效(综合医院中西医协同体制建设)。
关键词
幼年特发性关节炎
肺损伤
肺间质病变
肺部高分辨CT
肺功能
Juvenile idiopathic arthritis
Pulmonary injury
Interstitial lung disease
High resolution CT of the lung
Lung function
