High-Resolution Computed Tomography and Survival in Fibrosing Alveolitis

Abstract

High-resolution computed tomography findings were reviewed in 32 patients with cryptogenic fibrosing alveolitis and 18 with fibrosing alveolitis associated with connective tissue diseases (other than scleroderma). The percentage of abnormal lung, total ground-glass attenuation, ground-glass attenuation not associated with fibrosis, and fibrosis (reticular pattern and honeycombing) were compared with lung function and survival. In cryptogenic fibrosing alveolitis, 1-year mortality tended to be higher in patients with predominantly fibrotic lesions (39%) compared to mainly ground-glass attenuation (11%). Similar results were obtained in fibrosing alveolitis associated with connective tissue disease (1-year mortality 44% for fibrosis versus 22% for ground-glass attenuation), but the differences were not statistically significant. In cryptogenic fibrosing alveolitis, the extent of abnormal lung on initial computed tomography was 67% ± 20% in survivors at 1 year and 86% ± 8% in nonsurvivors (p < 0.005); this difference was still significant at 4 years. In fibrosing alveolitis associated with connective tissue disease, the degree of lung involvement between survivors and nonsurvivors was different at 1 year only, thus other factors seem to determine survival. Ground-glass attenuation associated with fibrosis adversely affected survival in cryptogenic fibrosing alveolitis, in contrast to isolated ground-glass attenuation.