儿童特发性间质性肺炎6例临床分析

Clinical characteristics of 6 children with idiopathic interstitial pneumonia

目的分析6例儿童特发性间质性肺炎(IIP)的临床特点和预后转归。方法回顾性分析2015年1月至2020年3月在南京医科大学附属儿童医院住院的6例IIP患儿的临床表现、辅助检查、治疗方法以及预后转归等信息。结果6例患儿中男2例、女4例,起病年龄4.8~10.6岁。6例患儿均为亚急性起病,症状均为咳嗽、气促伴发绀。6例患儿肺高分辨CT(HRCT)均为两肺弥漫性片絮影,其中2例伴有网格状影。5例患儿的肺功能为中至重度混合性通气功能障碍。4例患儿行肺活检病理检查。6例患儿的治疗药物均为全身糖皮质激素,其中2例联合吸入糖皮质激素。4例患儿为隐源性机化性肺炎(COP),其肺HRCT恢复正常的时间为1~11个月。2例患儿为非特异性间质性肺炎(NSIP),其肺HRCT残留纤维化病灶,有剧烈运动不耐受。6例患儿停用全身激素后随访1年至6年5个月,均无复发。结论儿童IIP的临床特点为亚急性起病,以咳嗽、气促伴发绀为症状,肺HRCT示两肺弥漫性片絮影,常见类型为COP和NSIP。儿童IIP全身糖皮质激素治疗有效,总体预后良好。

Objective To analyze the clinical characteristics and prognosis of 6 children with idiopathic interstitial pneumonia(IIP).Methods This retrospective study analyzed the clinical manifestations,examinations,treatment and prognosis of 6 children with IIP who were hospitalized in Children′s Hospital of Nanjing Medical University from January 2015 to March 2020.Results Of the 6 children,2 were males and 4 were females,aged 4.8 to10.6 years.All children had a subacute onset,and presented with cough,shortness of breath and cyanosis.The lung high-resolution CT(HRCT)showed diffuse patchiness in bilateral lung fields in all the children and reticular pattern in 2 cases.Pulmonary function test found moderate to severe mixed defect in 5 children.Lung biopsy was performed in 4 children.All of the 6 children were treated with systemic glucocorticoids,of whom 2 cases had additional inhaled glucocorticoids.Four children were finally diagnosed as cryptogenic organizing pneumonia(COP),whose lung HRCT return to normal in 1-11 months.Two children were finally diagnosed as nonspecific interstitial pneumonia(NSIP),and had long-term residual fibrosis on lung HRCT.The 6 children were followed up for 1 year to 6 years and 5 months after discontinuation of systemic glucocorticoids,and all had no recurrence.Conclusions The clinical characteristics of IIP in children are subacute onset presented with cough,shortness of breath,cyanosis and diffuse patchiness in bilateral lungs on HRCT.The common subtypes of IIP in children are COP and NSIP.Systemic glucocorticoid is effective for IIP in children and there is a good prognosis overall.