Risk factors for progression of structural lung disease in school-age children with cystic fibrosis

Background: Computed tomography (CT) is used to monitor progression of structural lung disease (SLD) in children with cystic fibrosis (CF). Our goals were to identify the risk factors for the annual progression of SLD and the impacts of airway pathogens on SLD. Method: Seventy-five school-aged children diagnosed with CF underwent 200 CT scans at Gothenburg CF Centre in the period 2003-2015. SLD was evaluated with a quantitative scoring system. Mixed models were used to calculate the yearly progression rates of SLD and FEV1 and to analyse the effects of common airway pathogens in CF. Results: The yearly mean progression (95% CI) rates for total disease (%Dis), bronchiectasis (%Be), and FEV1 were 0.62 (0.38-0.86), 0.43 (0.28-0.58) and -0.16 (-0.18-0.13), respectively. Adjusting for airway pathogens, the yearly mean progression rates for %Dis, %Be and FEV1 were 0.23 (-0.04-0.51), 0.12 (0.00-0.25), and -0.12 (-0.16-0.08), respectively. A single infection with P aeruginosa was associated with significant increase in lung damage, assessed as %Dis (p=0.044) and%Be (p=0.0047), but not in FEV1 (p=0.96). At age of 7 years, there was a good correlation between the extent of SLD and subsequent progression of %Dis (r=0.63, p=0.0042) and %Be (r=0.74, p=0.0057) while there was no significant correlation between the FEV1 and the rate of decline of FEV1 (r = -0.22, p=0.12). Conclusion: Intermittent respiratory infections with P aeruginosa were associated with significant SLD but no change in FEV1. More SLD at the age of 7 years signals a higher progression rate of SLD subsequently. (C) 2019 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.