Rationale: In cystic fibrosis (CF), lung disease is the predominant cause of morbidity and mortality. Little is known about the spectrum of structural abnormalities on CT scans from patients with CF with severe advanced lung disease (SALD). No specific CT scoring system for SALD is available.
Objectives: To design a quantitative CT scoring system for SALD, to determine the spectrum of structural abnormalities in patients with SALD and to correlate the SALD system with an existing scoring system for mild CF lung disease and pulmonary function tests (PFTs).
Methods: 57 patients with CF contributed one CT made during screening for lung transplantation. For the SALD system, lung tissue was divided into four components: infection/inflammation (including bronchiectasis, airway wall thickening, mucus and consolidations), air trapping/hypoperfusion, bulla/cysts and normal/hyperperfused tissue. The volume proportion of the components was estimated on a 0–100% scale; mean volumes for the whole lung were computed. Scores were correlated with Brody-II scores and PFTs.
Results: The SALD system identified a wide spectrum of structural abnormalities ranging from predominantly infection/inflammation to predominantly air trapping/hypoperfusion. SALD infection/inflammation scores correlated with Brody-II scores (rs = 0.36–0.64) and SALD normal/hyperperfusion scores correlated with forced expiratory volume in 1 s (FEV1; rs = 0.37). Reproducibility for both systems was good.
Conclusions: A CT scoring system was developed to characterise the structural abnormalities in patients with SALD. A wide spectrum was observed in SALD, ranging from predominantly air trapping to predominantly infection/inflammation-related changes. This spectrum may have clinical implications for patients with SALD.
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Funding This study was supported by grants from the Sophia CF research fund; the CF Trust of the Royal Children’s Hospital, Melbourne, Australia; the Dutch Cystic Fibrosis Foundation (NCFS); and the Italian CF Fund (IERFC). None of the sponsors was involved in the study design, data collection, analysis, interpretation of data, writing of the report, or in the decision to submit the paper for publication.
Competing interests None.
Provenance and Peer review Not commissioned; externally peer reviewed.
Ethics approval The review boards of all three participating centres approved the study protocol and waived informed consent.
▸ Additional figures, details of patients and methods are published online only at http://thorax.bmj.com/content/vol64/issue10
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