儿童特发性肺纤维化临床特征、远期预后及肺功能改变

Analysis of the clinical characteristics, long-term prognosis and changes of pulmonary function in children with idiopathic pulmonary fibrosis

目的探讨儿童特发性肺纤维化(IPF)的临床特征、远期预后及肺功能改变。方法回顾性分析2008年1月至2018年12月重庆医科大学附属儿童医院呼吸中心28例IPF患儿的临床资料,随访远期预后及肺功能改变。结果28例患儿年龄为0.5~15.7岁(中位数3.9岁)。临床表现为咳嗽(28例,100.0%)、气促(25例,89.3%)、发绀(19例,67.9%)、呼吸困难(11例,39.3%),Velcho啰音(12例,42.9%)、吸气性三凹征(11例,39.3%)、杵状指/趾(6例,21.4%)、呼吸音降低(5例,17.9%)。胸部高分辨CT(HRCT)可见网格状影、不规则囊状透光影呈蜂窝状改变及致密影,部分(7例)肺间质气肿/肺气肿/纵隔气肿。3例肺活检为肺泡腔及肺泡间隔纤维组织增生、实变,肺泡壁增厚及新老病变共存。本组中4例因其他原因未行药物治疗(3例放弃治疗,1例住院期间因呼吸循环衰竭死亡),24例分别予口服泼尼松、N-乙酰半胱氨酸和阿奇霉素任一单药或联合治疗,11例出院时症状好转,13例症状无改善。24例继续按原方案口服药物治疗,8例随访胸部影像学3个月~4年,7例胸部HRCT病变较前相仿,1例胸部HRCT病变较前增多;电话随访2~7年,最长用药时间4年,12例失访,7例仍运动受限,3例死亡,2例无临床症状。3例随访肺功能2~3年,其中2例肺功能较前降低;1例用力肺活量占预测值的百分比和呼气末峰流速占预测值的百分比较前好转,但第1秒用力呼气容积占预测值的百分比较前下降。结论儿童IPF临床表现缺乏特异性,胸部HRCT对IPF诊断、初步监测病变活动情况具有重要价值,远期随访部分患儿临床症状好转,肺功能多降低,部分指标可改善。

Objective To investigate the clinical characteristics,long-term prognosis and changes of pulmonary function in children with idiopathic pulmonary fibrosis(IPF).Methods The clinical data,long-term prognosis and changes of pulmonary function of children with IPF admitted to the Department of Pediatric Respiratory Center in Children′s Hospital of Chongqing Medical University from January 2008 to December 2018 were retrospectively analyzed.Results A total of 28 cases were included,with the median age of 3.9 years(range:0.5 to 15.7 years).Cough(28 cases,100.0%),tachypnea(25 cases,89.3%),cyanosis(19 cases,67.9%),dyspnea(11 cases,39.3%),Velcho rales(12 cases,42.9%),inspiratory three concave sign(11 cases,39.3%),clubbed fingers and toes(6 cases,21.4%)and diminished breath sounds(5 cases,17.9%)were main clinical manifestations.Chest high-resolution computed tomography(HRCT)mainly displayed grid shadow,irregular sac-like light-transparent shadow with ho-neycomb changes and dense shadow,partial(7 cases)pulmonary interstitial emphysema/emphysema/pneumomediastinum.Three cases of lung biopsy showed hyperplasia and consolidation of alveolar space and alveolar septal fibrosis,thickening of alveolar wall and coexistence of new and old lesions.In this group,4 cases did not receive drug therapy due to other reasons(3 cases abandoned therapy,and 1case died of respiratory and circulatory failure during hospitalization).Twenty-four cases were treated with single or combination of oral Prednisone,N-acetylcysteine and Azithromycin.Eleven cases had improved symptoms when discharged,and 13 cases showed no improvement.Twenty-four cases continued to receive oral medication therapy according to the original protocol.Eight cases were followed up for chest HRCT for 3 months to 4 years,the chest HRCT lesions of 7 cases were similar to before,and those of 1 case increased than before.All cases received telephone follow-up for 2 to 7 years;the maximum duration of medication was 4 years.Twelve cases were lost to follow-up,7 cases had motion limitation,3 cases died,and 2 cases had no clinical symptom.Three cases were followed up for pulmonary function for 2 to 3 years,among which 2 cases had pulmonary function decreased than before;1 case had improvements in forced vital capacity as a percentage of the predicted value and peak expiratory flow as a percentage of the predicted value,but decline in forced expiratory volume in the first se-cond as a percentage of the predicted value.Conclusions The clinical manifestations of children with IPF are lack of specificity.Chest HRCT is of great value in the diagnosis of IPF and preliminary monitoring of the activity of lesion.In the long-term follow-up,some of cases have improvements in symptoms;pulmonary function mostly decreases,but part of indexes can be improved.