Thin-Section CT in Patients with Cystic Fibrosis: Correlation with Peak Exercise Capacity and Body Mass Index

Purpose: To evaluate whether thin-section chest computed tomographic (CT) findings correlate with exercise capacity, body mass index (BMI), dyspnea, and leg discomfort in patients with cystic fibrosis (CF).

Materials and Methods: Institutional ethics committee approval was obtained, and patients provided written informed consent. Twenty-two patients (13 male and nine female patients; mean age, 22 years ± 5.9; age range, 17–41 years) with stable CF underwent thin-section CT and exercise testing on a cycle ergometer. Three radiologists blinded to the clinical severity of disease and the spirometric findings of all patients independently and randomly scored all scans with a modified Bhalla scoring system. The primary measurement of the outcome of exercise testing was percentage of predicted peak O2 uptake. Univariate (Spearman rank correlation) and multivariate analyses were used to compare thin-section CT, clinical (age, sex, spirometric data, and BMI), and exercise measurements.

Results: The correlation between total thin-section CT score and percentage of predicted peak O2 uptake was stronger than the correlation between the percentage of predicted peak O2 uptake and any clinical measurement (R = −0.60, P < .01). The thin-section CT structural abnormalities that had the strongest correlation with percentage of predicted peak O2 uptake were severity of bronchiectasis and presence of sacculations or abscesses (R = −0.70 and −0.71, respectively; P < .01). Multivariate analysis showed total thin-section CT score to be the only significant predictor of exercise capacity, accounting for 42% of the variance in percentage of predicted peak O2 uptake.

Conclusion: In patients with CF, the correlation between thin-section CT score and exercise limitation is stronger than that between spirometry results or BMI and exercise limitation.

© RSNA, 2006

References

  • 1 Devaney J, Glennon M, Farrell G, et al. Cystic fibrosis mutation frequencies in an Irish population. Clin Genet 2003; 63: 121–125. Crossref, MedlineGoogle Scholar
  • 2 Jaffe A, Bush A. Cystic fibrosis: review of the decade. Monaldi Arch Chest Dis 2001; 56: 240–247. MedlineGoogle Scholar
  • 3 Brody AS, Klein JS, Molina PL, Quan J, Bean JA, Wilmott RW. High-resolution computed tomography in young patients with cystic fibrosis: distribution of abnormalities and correlation with pulmonary function tests. J Pediatr 2004; 145: 32–38. Crossref, MedlineGoogle Scholar
  • 4 Brody AS, Molina PL, Klein JS, Rothman BS, Ramagopal M, Swartz DR. High-resolution computed tomography of the chest in children with cystic fibrosis: support for use as an outcome surrogate. Pediatr Radiol 1999; 29: 731–735. Crossref, MedlineGoogle Scholar
  • 5 de Jong PA, Nakano Y, Lequin MH, et al. Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis. Eur Respir J 2004; 23: 93–97. Crossref, MedlineGoogle Scholar
  • 6 de Jong PA, Ottink MD, Robben SG, et al. Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements. Radiology 2004; 231: 434–439. LinkGoogle Scholar
  • 7 Bonnel AS, Song SM, Kesavarju K, et al. Quantitative air-trapping analysis in children with mild cystic fibrosis lung disease. Pediatr Pulmonol 2004; 38: 396–405. Crossref, MedlineGoogle Scholar
  • 8 Helbich TH, Heinz-Peer G, Fleischmann D, et al. Evolution of CT findings in patients with cystic fibrosis. AJR Am J Roentgenol 1999; 173: 81–88. Crossref, MedlineGoogle Scholar
  • 9 Helbich TH, Heinz-Peer G, Eichler I, et al. Cystic fibrosis: CT assessment of lung involvement in children and adults. Radiology 1999; 213: 537–544. LinkGoogle Scholar
  • 10 Dorlochter L, Helgheim V, Roksund OD, Rosendahl K, Fluge G. Shwachman-Kulczyski score and resting energy expenditure in cystic fibrosis. J Cyst Fibros 2003; 2: 148–151. Crossref, MedlineGoogle Scholar
  • 11 Bradley J, McAlister O, Elborn S. Pulmonary function, inflammation, exercise capacity and quality of life in cystic fibrosis. Eur Respir J 2001; 17: 712–715. Crossref, MedlineGoogle Scholar
  • 12 de Jong W, Kaptein AA, van der Schans CP, et al. Quality of life in patients with cystic fibrosis. Pediatr Pulmonol 1997; 23: 95–100. Crossref, MedlineGoogle Scholar
  • 13 Nixon PA, Orenstein DM, Kelsey SF, Doershuk CF. The prognostic value of exercise testing in patients with cystic fibrosis. N Engl J Med 1992; 327: 1785–1788. Crossref, MedlineGoogle Scholar
  • 14 Pianosi P, Leblanc J, Almudevar A. Peak oxygen uptake and mortality in children with cystic fibrosis. Thorax 2005; 60: 50–54. Crossref, MedlineGoogle Scholar
  • 15 Prasad SA, Cerny FJ. Factors that influence adherence to exercise and their effectiveness: application to cystic fibrosis. Pediatr Pulmonol 2002; 34: 66–72. Crossref, MedlineGoogle Scholar
  • 16 American Thoracic Society; American College of Chest Physicians. ATS/ACCP statement on cardiopulmonary exercise testing. Am J Respir Crit Care Med 2003; 167: 211–277. Crossref, MedlineGoogle Scholar
  • 17 Marcotte JE, Grisdale RK, Levison H, Coates AL, Canny GJ. Multiple factors limit exercise capacity in cystic fibrosis. Pediatr Pulmonol 1986; 2: 274–281. Crossref, MedlineGoogle Scholar
  • 18 Klijn PH, van der Net J, Kimpen JL, Helders PJ, van der Ent CK. Longitudinal determinants of peak aerobic performance in children with cystic fibrosis. Chest 2003; 124: 2215–2219. Crossref, MedlineGoogle Scholar
  • 19 Bhalla M, Turcios N, Aponte V, et al. Cystic fibrosis: scoring system with thin-section CT. Radiology 1991; 179: 783–788. LinkGoogle Scholar
  • 20 Austin JH, Muller NL, Friedman PJ, et al. Glossary of terms for CT of the lungs: recommendations of the Nomenclature Committee of the Fleischner Society. Radiology 1996; 200: 327–331. LinkGoogle Scholar
  • 21 Webb WR, Stern EJ, Kanth N, Gamsu G. Dynamic pulmonary CT: findings in healthy adult men. Radiology 1993; 186: 117–124. LinkGoogle Scholar
  • 22 McKone EF, Barry SC, FitzGerald MX, Gallagher CG. Reproducibility of maximal exercise ergometer testing in patients with cystic fibrosis. Chest 1999; 116: 363–368. Crossref, MedlineGoogle Scholar
  • 23 Borg GA. Psychophysical bases of perceived exertion. Med Sci Sports Exerc 1982; 14: 377–381. MedlineGoogle Scholar
  • 24 Crapo RO, Hankinson JL, Irvin C, MacIntyre NR, Voter KZ, Wise RA. American Thoracic Society statement: standardization of spirometry, 1994 update. Am J Respir Crit Care Med 1995; 152: 1107–1136. Crossref, MedlineGoogle Scholar
  • 25 Quanjer PH, Tammeling GJ, Cotes JE, Pedersen OF, Peslin R, Yernault JC. Lung volumes and forced ventilatory flows: Report Working Party Standardization of Lung Function Tests, European Community for Steel and Coal—official statement of the European Respiratory Society. Eur Respir J Suppl 1993; 16: 5–40. Crossref, MedlineGoogle Scholar
  • 26 Jones N. Clinical exercise testing. Philadelphia, Pa: Saunders, 1988. Google Scholar
  • 27 Lynch DA, Brasch RC, Hardy KA, Webb WR. Pediatric pulmonary disease: assessment with high-resolution ultrafast CT. Radiology 1990; 176: 243–248. LinkGoogle Scholar
  • 28 Hansell DM, Strickland B. High-resolution computed tomography in pulmonary cystic fibrosis. Br J Radiol 1989; 62: 1–5. Crossref, MedlineGoogle Scholar
  • 29 Robinson TE, Leung AN, Northway WH, et al. Spirometer-triggered high-resolution computed tomography and pulmonary function measurements during an acute exacerbation in patients with cystic fibrosis. J Pediatr 2001; 138: 553–559. Crossref, MedlineGoogle Scholar
  • 30 Robinson TE, Leung AN, Northway WH, et al. Composite spirometric-computed tomography outcome measure in early cystic fibrosis lung disease. Am J Respir Crit Care Med 2003; 168: 588–593. Crossref, MedlineGoogle Scholar
  • 31 Demirkazik FB, Ariyurek OM, Ozcelik U, Gocmen A, Hassanabad HK, Kiper N. High resolution CT in children with cystic fibrosis: correlation with pulmonary functions and radiographic scores. Eur J Radiol 2001; 37: 54–59. Crossref, MedlineGoogle Scholar
  • 32 Coates AL, Boyce P, Shaw DG, Godfrey S, Mearns M. Relationship between the chest radiograph, regional lung function studies, exercise tolerance, and clinical condition in cystic fibrosis. Arch Dis Child 1981; 56: 106–111. Crossref, MedlineGoogle Scholar
  • 33 Cerny FJ, Pullano TP, Cropp GJ. Cardiorespiratory adaptations to exercise in cystic fibrosis. Am Rev Respir Dis 1982; 126: 217–220. MedlineGoogle Scholar
  • 34 Thin AG, Dodd JD, Gallagher CG, Fitzgerald MX, McLoughlin P. Effect of respiratory rate on airway deadspace ventilation during exercise in cystic fibrosis. Respir Med 2004; 98: 1063–1070. Crossref, MedlineGoogle Scholar
  • 35 Tomashefski JF, Konstan MW, Bruce MC, Abramowsky CR. The pathologic characteristics of interstitial pneumonia in cystic fibrosis: a retrospective autopsy study. Am J Clin Pathol 1989; 91: 522–530. Crossref, MedlineGoogle Scholar
  • 36 Edwards EA, Narang I, Li A, Hansell DM, Rosenthal M, Bush A. HRCT lung abnormalities are not a surrogate for exercise limitation in bronchiectasis. Eur Respir J 2004; 24: 538–544. Crossref, MedlineGoogle Scholar
  • 37 Blau H, Mussaffi-Georgy H, Fink G, et al. Effects of an intensive 4-week summer camp on cystic fibrosis: pulmonary function, exercise tolerance, and nutrition. Chest 2002; 121: 1117–1122. Crossref, MedlineGoogle Scholar
  • 38 Shah RM, Sexauer W, Ostrum BJ, Fiel SB, Friedman AC. High-resolution CT in the acute exacerbation of cystic fibrosis: evaluation of acute findings, reversibility of those findings, and clinical correlation. AJR Am J Roentgenol 1997; 169: 375–380. Crossref, MedlineGoogle Scholar
  • 39 Baldwin DR, Hill AL, Peckham DG, Knox AJ. Effect of addition of exercise to chest physiotherapy on sputum expectoration and lung function in adults with cystic fibrosis. Respir Med 1994; 88: 49–53. Crossref, MedlineGoogle Scholar
  • 40 Steinkamp G, Wiedemann B. Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project. Thorax 2002; 57: 596–601. Crossref, MedlineGoogle Scholar
  • 41 Ionescu AA, Nixon LS, Luzio S, et al. Pulmonary function, body composition, and protein catabolism in adults with cystic fibrosis. Am J Respir Crit Care Med 2002; 165: 495–500. Crossref, MedlineGoogle Scholar
  • 42 Mayo JR, Aldrich J, Muller NL. Radiation exposure at chest CT: a statement of the Fleischner Society. Radiology 2003; 228: 15–21. LinkGoogle Scholar
  • 43 Brody AS. Scoring systems for CT in cystic fibrosis: who cares? [editorial]. Radiology 2004; 231: 296–298. LinkGoogle Scholar
  • 44 Pianosi P, Pelech A. Stroke volume during exercise in cystic fibrosis. Am J Respir Crit Care Med 1996; 153: 1105–1109. Crossref, MedlineGoogle Scholar
  • 45 Moser C, Tirakitsoontorn P, Nussbaum E, Newcomb R, Cooper DM. Muscle size and cardiorespiratory response to exercise in cystic fibrosis. Am J Respir Crit Care Med 2000; 162: 1823–1827. Crossref, MedlineGoogle Scholar
  • 46 de Meer K, Jeneson JA, Gulmans VA, van der Laag J, Berger R. Efficiency of oxidative work performance of skeletal muscle in patients with cystic fibrosis. Thorax 1995; 50: 980–983. Crossref, MedlineGoogle Scholar

Article History

Published in print: July 2006