The spectrum of structural abnormalities on CT scans from patients with CF with severe advanced lung disease

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.Objectiv...

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Bibliographic Details
Published in:Thorax 2009-10, Vol.64 (10), p.876-882
Main Authors: Loeve, M, van Hal, P Th W, Robinson, P, de Jong, P A, Lequin, M H, Hop, W C, Williams, T J, Nossent, G D, Tiddens, H A
Format: Article
Language:English
Subjects:
Adolescent
Adult
Biological and medical sciences
Cardiology. Vascular system
Cystic fibrosis
Cystic Fibrosis - diagnostic imaging
Cystic Fibrosis - pathology
Cystic Fibrosis - physiopathology
Cysts
Female
Forced Expiratory Volume - physiology
Histology
Humans
Hypotheses
Infections
Inflammation
Life expectancy
Lung - pathology
Lung diseases
Male
Medical imaging
Medical prognosis
Medical sciences
Morphology
Mortality
Observer Variation
Patients
Pneumology
Pneumonia - diagnostic imaging
Pneumonia - pathology
Pneumonia - physiopathology
Respiratory Tract Infections - diagnostic imaging
Respiratory Tract Infections - pathology
Respiratory Tract Infections - physiopathology
Retrospective Studies
Scanners
Studies
Tomography, X-Ray Computed
Transplants & implants
Vital Capacity - physiology
Young Adult
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Summary: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.
ISSN:0040-6376
1468-3296
DOI:10.1136/thx.2008.110908