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

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.