Background Childhood interstitial lung disease (chILD) encompasses a group of rare heterogeneous respiratory conditions associated with significant morbidity and mortality. Reports suggest that many patients diagnosed with chILD continue to have potentially progressive or fibrosing disease into adulthood. Over the last decade, the spectrum of conditions within chILD has widened substantially, with the discovery of novel entities through advanced genetic testing. However, most evidence is often limited to small case series, with reports disseminated across an array of subspecialty, clinical and molecular journals. In particular, the frequency, management and outcome of paediatric pulmonary fibrosis is not well characterised, unlike in adults, where clear diagnosis and treatment guidelines are available. Methods and results This review assesses the current understanding of pulmonary fibrosis in chILD. Based on registry data, we have provisionally estimated the occurrence of fibrosis in various manifestations of chILD, with 47 different potentially fibrotic chILD entities identified. Published evidence for fibrosis in the spectrum of chILD entities is assessed, and current and future issues in management of pulmonary fibrosis in childhood, continuing into adulthood, are considered. Conclusions There is a need for improved knowledge of chILD among pulmonologists to optimise the transition of care from paediatric to adult facilities. Updated evidence-based guidelines are needed that incorporate recommendations for the diagnosis and management of immune-mediated disorders, as well as chILD in older children approaching adulthood.

中文翻译：

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肺纤维化可能始于婴儿期：从儿童期到成人间质性肺病


背景 儿童间质性肺病 （chILD） 包括一组罕见的异质性呼吸系统疾病，与显着的发病率和死亡率相关。报告表明，许多被诊断患有 chILD 的患者在成年后仍可能患有进行性或纤维化疾病。在过去十年中，随着通过先进的基因检测发现新实体，chILD 的病症范围大幅扩大。然而，大多数证据通常仅限于小型病例系列，报告在一系列亚专业、临床和分子期刊上传播。特别是，小儿肺纤维化的频率、管理和结局没有得到很好的表征，这与成人不同，成人有明确的诊断和治疗指南。方法和结果 本综述评估了目前对 chILD 中肺纤维化的理解。根据登记数据，我们临时估计了 chILD 各种表现中纤维化的发生率，确定了 47 种不同的潜在纤维化 chILD 实体。评估了 chILD 实体谱系中纤维化的已发表证据，并考虑了儿童期肺纤维化管理的当前和未来问题，并持续到成年。结论 需要提高肺科医生对 chILD 的了解，以优化从儿科到成人设施的护理过渡。需要更新的循证指南，其中包括免疫介导性疾病的诊断和管理建议，以及接近成年的大龄儿童的 chILD。