Cystic fibrosis: CT assessment of lung involvement in children and adults

Radiology. 1999 Nov;213(2):537-44. doi: 10.1148/radiology.213.2.r99nv04537.


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.

Publication types

  • Clinical Trial
  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Child
  • Child, Preschool
  • Cystic Fibrosis / diagnostic imaging*
  • Female
  • Humans
  • Infant
  • Male
  • Prospective Studies
  • Tomography, X-Ray Computed*