Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Padova, Italy.
German Center for Lung Research, Hauner Children's Hospital, University of Munich, Munich, Germany.
Curr Opin Pulm Med. 2019 Sep;25(5):418-425. doi: 10.1097/MCP.0000000000000594.
Interstitial lung disease (ILD) consists of a large and heterogeneous group of disorders that are classified together because of similar clinical, radiographic, physiologic or pathologic manifestations. Overall, although there is overlap between adult and childhood ILD (chILD), there are many differences in disease causes and prevalences.
In the last few years, our understanding of adult ILD pathobiology has improved substantially. This is particularly true for idiopathic pulmonary fibrosis, the most common of the idiopathic interstitial pneumonias, wherein recently developed guideline documents provide recommendations for the diagnosis and clinical management of patients. For chILD, similar guidelines are yet to be developed. However, complications and long-term pulmonary outcomes of paediatric disease are better appreciated, which make the implementation of a successful transition program from paediatric to adult care an urgent need. Similarly important is the development of guidelines on performance and interpretation of genetic testing in affected and unaffected relatives of familial cases and in children of adult-onset ILD patients. Lung transplantation appears to be as successful as in adult patients for end-stage disease. Paediatric pulmonologists should engage with the adult multidisciplinary teams and benefit from their much more extensive experience.
Childhood and adult ILD share a number of aspects, which give children and adult ILD specialists exciting opportunities to collaborate and learn from each other. Such collaborative effort between child and adult ILD experts is crucial for successful future development in the field.
间质性肺疾病（ILD）由一大组异质性疾病组成，这些疾病因相似的临床、影像学、生理学或病理学表现而被归为一类。总体而言，尽管成人和儿童间质性肺疾病（chILD）有一定的重叠，但在疾病病因和流行率方面存在许多差异。
在过去的几年中，我们对成人ILD 病理生物学的理解有了实质性的提高。这在特发性肺纤维化中尤其如此，特发性肺纤维化是特发性间质性肺炎中最常见的一种，最近制定的指南文件为患者的诊断和临床管理提供了建议。对于 chILD，类似的指南尚未制定。然而，儿童疾病的并发症和长期肺部结局得到了更好的认识，这使得从儿科向成人护理成功过渡的计划成为当务之急。同样重要的是，制定针对家族性病例的受累和未受累亲属以及成人发病的 ILD 患儿的遗传检测的性能和解释的指南。肺移植在终末期疾病患者中与成人患者一样成功。儿科肺病专家应与成人多学科团队合作，并从他们更广泛的经验中受益。
儿童和成人间质性肺疾病有许多共同之处，这为儿童和成人间质性肺疾病专家提供了令人兴奋的合作和相互学习的机会。儿童和成人间质性肺疾病专家之间的这种合作对于该领域未来的成功发展至关重要。
