Pulmonary fibrosis may begin in infancy: from childhood to adult interstitial lung disease
Year: 2024
Type: review
Abstract: 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.
Source: Thorax
Institutions: LMU Klinikum, German Center for Lung Research, Ludwig-Maximilians-Universität München, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Children's Hospital Los Angeles, Children's Hospital Colorado, University of Colorado Denver, Inserm, Université Paris-Est Créteil, Fondation Maladies Rares, Hôpital Intercommunal de Créteil, Institut Mondor de Recherche Biomédicale, Sorbonne Université, Hôpital Armand-Trousseau, Assistance Publique – Hôpitaux de Paris, Maladies génétiques d’expression pédiatrique, Medizinische Hochschule Hannover, Children's Hospital of Philadelphia, Seattle Children's Hospital, University of Washington Medical Center
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Funder: Boehringer Ingelheim
OpenAlex ID: W4401664767