Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis

Pim A de Jong ¹, Anders Lindblad ², Lorenzo Rubin ³, Wim CJ Hop ⁴, Johan C de Jongste ¹, Mela Brink ⁵ and Harm AWM Tiddens ^1*

¹ Cystic Fibrosis Team Rotterdam, Erasmus MC-Sophia, Pediatric Pulmonology, Rotterdam, Netherlands
² The West Swedish CF centre, Queens Silvia Children's Hospital, Pediatric Pulmonology, Gothenborg, Sweden
³ Policlinico GB Rossi, Instituto di Radiologia, Verona, Italy
⁴ Erasmus MC-University, Epidemiology and Biostatistics, Rotterdam, Netherlands
⁵ The West Swedish CF centre, Queens Silvia Children's Hospital, Radiology, Gothenborg, Sweden

^* To whom correspondence should be addressed. E-mail: h.tiddens{at}erasmusmc.nl.

Accepted 12 October 2005

Abstract

Background: In a single centre study structural lung abnormalities on computed tomography (CT) scans worsened in children with cystic fibrosis (CF) who had on average stable pulmonary function tests (PFTs). The aims of this study were to compare the ability of CT-scores and PFTs to detect changes in CF lung disease in children and adults.

Methods: CT scans and PFTs were retrospectively studied in a cohort of CF-patients aged 5 to 52 years for whom 2 or 3 CT scans at 3-year intervals were available in combination with PFTs (FEV₁, FVC, FEV₁/FVC, MEF₂₅, MEF₅₀, RV, TLC and RV/TLC). All CT scans were scored by two observers. PFTs were expressed as percentage predicted and Z-score.

Results: Of 119 patients included, 92 patients had two and 24 had three CT scans. CT (composite and components) scores and PFTs both worsened significantly (p<0.02). Peripheral bronchiectasis worsened by 1.7% per year in children (p<0.0001) and by 1.5% per year in adults (p<0.0001). Bronchiectasis worsened in 68 of 92 patients relative to 54 of 92 for FEV₁ and it worsened in 27 patients with stable or improving FEV₁. CT-score and its components and PFTs showed similar worsening rates for adults and children (p>0.09).

Conclusion: Peripheral bronchiectasis CT-score worsens faster and more frequently than PFTs in children and adults with CF, which demonstrates that PFTs and CT measure different aspects of CF lung disease. Our data support previous findings that peripheral bronchiectasis CT-score has an added value to PFTs in monitoring CF lung disease.

Keywords: bronchiectasis, cystic fibrosis, lung structure, pulmonary function tests, tomography x-ray computed

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