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

Abstract

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.

Clinical course and risk factors for severe COVID-19 among Italian patients with cystic fibrosis: a study within the Italian Cystic Fibrosis Society

“…First, the follow-up was relatively short and only the short-term clinical consequences of SARS-CoV-2 infection could be described. Second, we measured only FEVp to evaluate the effects of SARS-CoV-2 infection on respiratory function; other more sensitive measures might provide different results, especially in younger patients who usually show FEVp values within the normal range [ 36 ]. Third, risk factors for severe COVID-19 were evaluated mostly in a population of cases presenting with symptomatic infection.…”

Burden of cystic fibrosis in children <12 years of age prior to the introduction of CFTR modulator therapies

“…8 30 These observations underscore the need for more sensitive and age-appropriate methods to assess lung disease in children. 31 …”

“…Importantly, spirometry is known to have limited sensitivity in evaluating early lung disease; other modalities, such as multiple breath washout (ie, lung clearance index), have demonstrated functional lung disease in those with normal ppFEV 1 8 30. These observations underscore the need for more sensitive and age-appropriate methods to assess lung disease in children 31…”

The evolving role of radiological imaging in cystic fibrosis

“…2) [1 ▪ ,5 ▪▪ ,12 ▪ ]. These are only available in specialized centres, however [4 ▪ ]. PRAGMA-CF has been found to be superior to CF-CT for assessing both early lung disease in children as well as more advanced disease in older patients [11 ▪ ].…”