Abstract：
BACKGROUND: Interstitial lung disease in children (chILD) are rare and mostly severe lung diseases. Very few epidemiological data are available in limited series of patients. The aim of this study was to assess the prevalence and incidence of chILD in France.
METHODS: We performed within the RespiRare network a multicentre retrospective observational study in patients with chILD from 2000 to 2022 and a prospective evaluation of chILD\'s incidence between February 2022 and 2023.
RESULTS: chILD was reported in 790 patients in 42 centres. The estimated 2022 prevalence in France was 44 /million children (95% CI 40.76 to 47.46) and the computed incidence was 4.4 /million children (95% CI 3.44 to 5.56). The median age at diagnosis was 3 months with 16.9% of familial forms. Lung biopsy and genetic analyses were performed in 23.4% and 76.9%, respectively. The most frequent chILD aetiologies in the <2 years group were surfactant metabolism disorders (16.3%) and neuroendocrine cell hyperplasia of infancy (11.8%), and in the 2-18 years group diffuse alveolar haemorrhage (12.2%), connective tissue diseases (11.4%), hypersensitivity pneumonitis (8.8%) and sarcoidosis (8.8%). The management included mainly oxygen therapy (52%), corticosteroid pulses (56%), oral corticosteroids (44%), azithromycin (27.2%), enteral nutrition (26.9%), immunosuppressants (20.3%) and hydroxychloroquine (15.9%). The 5-year survival rate was 57.3% for the patients diagnosed before 2 years and 86% between 2 and 18 years.
CONCLUSIONS: This large and systematic epidemiological study confirms a higher incidence and prevalence of chILD than previously described. In order to develop international studies, efforts are still needed to optimise the case collection and to harmonise diagnostic and management practices.
METHODS: We performed within the RespiRare network a multicentre retrospective observational study in patients with chILD from 2000 to 2022 and a prospective evaluation of chILD\'s incidence between February 2022 and 2023.
RESULTS: chILD was reported in 790 patients in 42 centres. The estimated 2022 prevalence in France was 44 /million children (95% CI 40.76 to 47.46) and the computed incidence was 4.4 /million children (95% CI 3.44 to 5.56). The median age at diagnosis was 3 months with 16.9% of familial forms. Lung biopsy and genetic analyses were performed in 23.4% and 76.9%, respectively. The most frequent chILD aetiologies in the <2 years group were surfactant metabolism disorders (16.3%) and neuroendocrine cell hyperplasia of infancy (11.8%), and in the 2-18 years group diffuse alveolar haemorrhage (12.2%), connective tissue diseases (11.4%), hypersensitivity pneumonitis (8.8%) and sarcoidosis (8.8%). The management included mainly oxygen therapy (52%), corticosteroid pulses (56%), oral corticosteroids (44%), azithromycin (27.2%), enteral nutrition (26.9%), immunosuppressants (20.3%) and hydroxychloroquine (15.9%). The 5-year survival rate was 57.3% for the patients diagnosed before 2 years and 86% between 2 and 18 years.
CONCLUSIONS: This large and systematic epidemiological study confirms a higher incidence and prevalence of chILD than previously described. In order to develop international studies, efforts are still needed to optimise the case collection and to harmonise diagnostic and management practices.
摘要：
背景：儿童间质性肺病（chILD）是罕见且大多为严重的肺部疾病。在有限的患者系列中，流行病学数据很少。这项研究的目的是评估法国chILD的患病率和发病率。
方法：我们在RespiRare网络中对2000年至2022年chILD患者进行了一项多中心回顾性观察性研究，并对2022年2月至2023年chILD的发病率进行了前瞻性评估。
结果：在42个中心的790名患者中报告了chILD。估计2022年法国的患病率为44/百万儿童（95％CI40.76至47.46），计算的发病率为4.4/百万儿童（95％CI3.44至5.56）。诊断时的中位年龄为3个月，有16.9％的家族形式。肺活检和遗传分析分别为23.4%和76.9%，分别。<2岁组最常见的child病因是表面活性剂代谢障碍（16.3％）和婴儿期神经内分泌细胞增生（11.8％），在2-18岁组弥漫性肺泡出血（12.2%），结缔组织病（11.4%），过敏性肺炎（8.8％）和结节病（8.8％）。管理主要包括氧疗（52%），皮质类固醇脉冲（56％），口服皮质类固醇（44%），阿奇霉素（27.2%），肠内营养（26.9%），免疫抑制剂（20.3％）和羟氯喹（15.9％）。在2年之前诊断的患者的5年生存率为57.3％，在2至18年之间为86％。
结论：这项大规模且系统的流行病学研究证实了chILD的发病率和患病率高于先前的描述。为了发展国际研究，仍然需要努力优化病例收集，并协调诊断和管理实践。
方法：我们在RespiRare网络中对2000年至2022年chILD患者进行了一项多中心回顾性观察性研究，并对2022年2月至2023年chILD的发病率进行了前瞻性评估。
结果：在42个中心的790名患者中报告了chILD。估计2022年法国的患病率为44/百万儿童（95％CI40.76至47.46），计算的发病率为4.4/百万儿童（95％CI3.44至5.56）。诊断时的中位年龄为3个月，有16.9％的家族形式。肺活检和遗传分析分别为23.4%和76.9%，分别。<2岁组最常见的child病因是表面活性剂代谢障碍（16.3％）和婴儿期神经内分泌细胞增生（11.8％），在2-18岁组弥漫性肺泡出血（12.2%），结缔组织病（11.4%），过敏性肺炎（8.8％）和结节病（8.8％）。管理主要包括氧疗（52%），皮质类固醇脉冲（56％），口服皮质类固醇（44%），阿奇霉素（27.2%），肠内营养（26.9%），免疫抑制剂（20.3％）和羟氯喹（15.9％）。在2年之前诊断的患者的5年生存率为57.3％，在2至18年之间为86％。
结论：这项大规模且系统的流行病学研究证实了chILD的发病率和患病率高于先前的描述。为了发展国际研究，仍然需要努力优化病例收集，并协调诊断和管理实践。
