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(Radiology. 1999;213:537-544.)
© RSNA, 1999


Thoracic Imaging

Cystic Fibrosis: CT Assessment of Lung Involvement in Children and Adults1

Thomas H. Helbich, MD, Gertraud Heinz-Peer, MD, Irmgard Eichler, MD, Patrick Wunderbaldinger, MD, Manfred Götz, MD, Claudia Wojnarowski, MD, Robert C. Brasch, MD and Christian J. Herold, MD

1 From the Departments of Radiology (T.H.H., G.H.P., P.W., C.J.H.) and Pediatrics (I.E., M.G., C.W.), University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna, Austria, and the Department of Radiology, University of California, San Francisco, Calif (R.C.B.). From the 1994 RSNA scientific assembly. Received November 10, 1998; revision requested December 29; revision received March 8, 1999; accepted April 15. Supported in part by the Ludwig-Boltzmann-Institute for Radiologic Tumor Research. T.H.H. supported in part by a grant from the Max Kade Foundation. Address reprint requests to T.H.H. (e-mail: Thomas.Helbich @akhwien.ac.at).

PURPOSE: To compare a computed tomographic (CT)-based scoring system with nonimaging indexes of pulmonary status in patients with cystic fibrosis.

MATERIALS AND METHODS: Pulmonary CT findings were assessed in 117 patients with cystic fibrosis, with cases classified according to three groups by age; 0–5 years, 6–16 years, and 17 years and older. Images were examined for specific abnormalities, and the severity and anatomic extent of each sign were used to generate a score. Scores in each category and the global score for each patient were correlated with pulmonary function test results, clinical status, serum immunoglobulin levels, and genotype, all obtained within 2 weeks of CT.

RESULTS: The most frequent individual CT abnormalities were bronchiectasis in 94 (80.3%), peribronchial wall thickening in 89 (76.1%), mosaic perfusion in 71 (63.9%), and mucous plugging in 56 (51.3%) patients. The percentage of patients with specific CT findings and the overall CT scores increased significantly (P < .05) with progressively increasing age groups. All CT findings and the overall CT scores correlated significantly (P < .05) with the pulmonary function test results, serum immunoglobulin levels, and clinical scores. No relationship was observed between genotype and CT scores.

CONCLUSION: Scoring of CT studies in patients with cystic fibrosis seems to offer a reliable way to monitor disease status and progression and may provide a reasonable tool to assess treatment interventions.

Index terms: Bronchi, CT, 60.1211 • Bronchiectasis, 60.26 • Children, respiratory system, 60.252 • Emphysema, pulmonary, 60.751 • Fibrosis, cystic, 60.252 • Lung, CT, 60.1211 • Lung, interstitial disease, 60.252, 60.26, 60.751, 60.756, 60.76




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