Journal Article

Pulmonary fibrosis may begin in infancy: from childhood to adult interstitial lung disease

Griese, Matthias, Young, Lisa R, Epaud, Ralph, Deutsch, Gail H, Nathan, Nadia, Weinman, Jason P, Deterding, Robin R, Schwerk, Nicolaus, Warburton, David, Kurland, Geoffrey, Cidon, Michal

2024

Overview

BackgroundChildhood 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 resultsThis 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.ConclusionsThere 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.

• Publisher: BMJ Publishing Group Ltd and British Thoracic Society,BMJ Publishing Group LTD,BMJ Publishing Group
• Subject: Adenosine triphosphate / Adult / Adults / Biopsy / Child / Childhood / Children & youth / Childrens health / Connective tissue disease associated lung disease / Cysts / Drug induced Lung Disease / Genetic testing / Histology / Humans / Infant / Interstitial Fibrosis / Interstitial Lung Disease / Investigations / Lung diseases / Lung Diseases, Interstitial - diagnosis / Lung Diseases, Interstitial - etiology / Lung Diseases, Interstitial - therapy / Mutation / Paediatric interstitial lung disease / Paediatric Lung Disaese / Pediatrics / Pneumonia / Postpartum period / Pulmonary fibrosis / Pulmonary Fibrosis - diagnosis / Pulmonary Fibrosis - therapy / Pulmonology / Rare lung diseases / Smooth muscle / State of the Art Review / Surfactant protein / Surfactants / Systemic disease and lungs