4.3 Review

A multimodal approach to detect and monitor early lung disease in cystic fibrosis

Journal

EXPERT REVIEW OF RESPIRATORY MEDICINE
Volume 15, Issue 6, Pages 761-772

Publisher

TAYLOR & FRANCIS LTD
DOI: 10.1080/17476348.2021.1908131

Keywords

Cystic fibrosis; lung clearance index; magnetic resonance imaging

Funding

  1. Vertex Pharmaceuticals (Europe) Ltd.

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The early stages of lung involvement in cystic fibrosis can be asymptomatic, making early detection challenging. Currently, methods such as LCI, CT, and MRI are used to monitor the progression of lung disease and evaluate responses to interventions. Collaboration between pulmonology and radiology is crucial for improving care and outcomes in young CF patients.
Introduction: In the early stages, lung involvement in cystic fibrosis (CF) can be silent, with disease progression occurring in the absence of clinical symptoms. Irreversible airway damage is present in the early stages of disease; however, reliable biomarkers of early damage due to inflammation and infection that are universally applicable in day-to-day patient management have yet to be identified. Areas covered: At present, the main methods of detecting and monitoring early lung disease in CF are the lung clearance index (LCI), computed tomography (CT), and magnetic resonance imaging (MRI). LCI can be used to detect patients who may require more intense monitoring, identify exacerbations, and monitor responses to new interventions. High-resolution CT detects structural alterations in the lungs of CF patients with the best resolution of current imaging techniques. MRI is a radiation-free imaging alternative that provides both morphological and functional information. The role of MRI for short-term follow-up and pulmonary exacerbations is currently being investigated. Expert opinion: The roles of LCI and MRI are expected to expand considerably over the next few years. Meanwhile, closer collaboration between pulmonology and radiology specialties is an important goal toward improving care and optimizing outcomes in young patients with CF.

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