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. 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.

[1]  M. Stanton,et al.  Cystic fibrosis mortality and survival in the UK: 1947–2003 , 2007, European Respiratory Journal.

[2]  P. Saldiva,et al.  Accuracy of high resolution CT in assessing idiopathic pulmonary fibrosis histology by objective morphometric index. , 2002, Pathology, research and practice.

[3]  W. Webb,et al.  Air trapping on CT of patients with pulmonary embolism. , 2002, AJR. American journal of roentgenology.

[4]  M. Boyle.  Adult cystic fibrosis. , 2007, JAMA.

[5]  Pim A de Jong,et al.  Cystic fibrosis specific computed tomography scoring. , 2007, Proceedings of the American Thoracic Society.

[6]  L. Olson,et al.  Chronic pulmonary thromboembolism: detection of regional hypoperfusion with CT. , 1994, Radiology.

[7]  W. Hop,et al.  Cartilaginous airway wall dimensions and airway resistance in cystic fibrosis lungs. , 2000, The European respiratory journal.

[8]  N. Kaneko,et al.  Chronic Pulmonary Thromboembolism: Air Trapping on Computed Tomography and Correlation with Pulmonary Function Tests , 2003, Journal of computer assisted tomography.

[9]  H. Tiddens.  Chest computed tomography scans should be considered as a routine investigation in cystic fibrosis. , 2006, Paediatric respiratory reviews.

[10]  T. Liou,et al.  Selection of patients with cystic fibrosis for lung transplantation , 2002, Current opinion in pulmonary medicine.

[11]  Margaret Rosenfeld,et al.  Developing cystic fibrosis lung transplant referral criteria using predictors of 2-year mortality. , 2002, American journal of respiratory and critical care medicine.

[12]  H. Goo,et al.  Detection of air trapping on inspiratory and expiratory phase images obtained by 0.3-second cine CT in the lungs of free-breathing young children. , 2006, AJR. American journal of roentgenology.

[13]  M. Goris,et al.  Dornase alfa reduces air trapping in children with mild cystic fibrosis lung disease: a quantitative analysis. , 2005, Chest.

[14]  W. Hop,et al.  Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis , 2005, Thorax.

[15]  L. Greco,et al.  Cystic fibrosis: when should high-resolution computed tomography of the chest Be obtained? , 1998, Pediatrics.

[16]  J. Mayo,et al.  Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis , 2004, European Respiratory Journal.

[17]  J. Fleiss.  The design and analysis of clinical experiments , 1987 .

[18]  S. Xie,et al.  Risk factors for death of patients with cystic fibrosis awaiting lung transplantation. , 2006, American journal of respiratory and critical care medicine.

[19]  F. Fedele,et al.  Outcome of patients with cystic fibrosis awaiting lung transplantation. , 2000, American journal of respiratory and critical care medicine.

[20]  H. Tiddens,et al.  Monitoring cystic fibrosis lung disease in clinical trials: is it time for a change? , 2007, Proceedings of the American Thoracic Society.

[21]  W. Travis,et al.  Thin-walled cavities, cysts, and pneumothorax in Pneumocystis carinii pneumonia: further observations with histopathologic correlation. , 1990, Radiology.

[22]  V. Starnes,et al.  Clinical findings and lung pathology in children with cystic fibrosis. , 2002, American journal of respiratory and critical care medicine.

[23]  F. Long.  High-resolution computed tomography of the lung in children with cystic fibrosis: technical factors. , 2007, Proceedings of the American Thoracic Society.

[24]  H. Tiddens,et al.  Update on the application of chest computed tomography scanning to cystic fibrosis , 2006, Current opinion in pulmonary medicine.

[25]  P. Woodruff,et al.  Structural changes to airway smooth muscle in cystic fibrosis , 2005, Thorax.

[26]  P. Molina,et al.  High-resolution computed tomography in young patients with cystic fibrosis: distribution of abnormalities and correlation with pulmonary function tests. , 2004, The Journal of pediatrics.

[27]  C. Fracchia,et al.  Risk factors for early death in patients awaiting heart-lung or lung transplantation: experience at a single European center. , 1998, Transplantation.

[28]  P. Cullinan,et al.  Improving rate of decline of FEV1 in young adults with cystic fibrosis , 2005, Thorax.

[29]  P. Davis,et al.  Cystic fibrosis: a review of epidemiology and pathobiology. , 2007, Clinics in chest medicine.

[30]  D. McCauley,et al.  Cystic fibrosis: scoring system with thin-section CT. , 1991, Radiology.

[31]  M. Corey,et al.  Prediction of mortality in patients with cystic fibrosis. , 1992, The New England journal of medicine.

[32]  Charles A. Johnson,et al.  Effects of 12-Week Administration of Dornase Alfa in Patients with Advanced Cystic Fibrosis Lung Disease , 1996 .

[33]  A. Brody.  Cystic fibrosis: when should high-resolution computed tomography of the chest be obtained? , 1998, Pediatrics.

[34]  S. Sakai,et al.  Lung Cancer Associated With Diffuse Pulmonary Fibrosis: CT–Pathologic Correlation , 2003, Journal of thoracic imaging.

[35]  R. Viswanathan,et al.  Pathogenesis of Bronchiectasis , 1941, The Indian medical gazette.

[36]  K. McCoy,et al.  Effects of 12-week administration of dornase alfa in patients with advanced cystic fibrosis lung disease. Pulmozyme Study Group. , 1996, Chest.

[37]  W. Hop,et al.  Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements. , 2004, Radiology.

[38]  R. Novick,et al.  Effect of diagnosis on survival benefit of lung transplantation for end-stage lung disease , 1998, The Lancet.