To retrospectively assess the change in disease pattern of nonspecific interstitial pneumonia (NSIP) and idiopathic pulmonary fibrosis (IPF) findings seen at thin-section computed tomography (CT) at long-term follow-up and to compare the same with initial findings at CT. The study included 48 patients (28 men, 20 women; mean age, 57.5 years) with biopsy-proved NSIP (n = 23) or IPF (n = 25) who underwent CT at initial diagnosis and at follow-up 34-155 months later. The CT scans were randomized and reviewed by two independent thoracic radiologists for pattern and distribution of ground-glass opacity (GGO), reticulation, traction bronchiectasis and bronchiolectasis, and honeycombing. Statistical analysis was performed by using nonparametric methods and univariate logistic regression. Follow-up CT in patients with NSIP showed marked decrease in the extent of GGO, increase in reticulation, and a greater likelihood of peripheral distribution (all P < .05). At presentation, the CT findings were interpreted as suggestive of NSIP in 18 of 23 patients with NSIP and indeterminate or suggestive of IPF in five. In five (28%) of 18 patients with initial findings suggestive of NSIP, the follow-up CT scans were interpreted as more suggestive of IPF. No CT features seen at presentation allowed distinction between patients with NSIP that maintained an NSIP pattern at follow-up and those that progressed to an IPF pattern. At follow-up CT, 28% of patients with initial CT findings suggestive of NSIP progressed to findings suggestive of IPF. Similar initial CT findings for NSIP may have different imaging outcomes.