To estimate whether the lung abnormalities seen in collagen vascular diseases (CVD) were similar or distinct to those seen in idiopathic pulmonary fibrosis (IPF), and to ascertain whether the extent of the abnormalities on high-resolution CT (HRCT) correlated with pulmonary function, we reviewed HRCT findings and pulmonary function test results of 64 patients with either CVD (n = 55) or IPF (n = 9). Response to corticosteroid treatment was also evaluated in 20 of the 64. High incidence of honeycomb lesion was observed in IPF (9/9, 100%) and in progressive systemic sclerosis (PSS) (11/14, 79%). CVD, except for PSS, had a low incidence of honeycomb lesion (27%). On the other hand, incidence of ground-glass shadow in CVD (47/55, 85%) was the same as that in IPF (8/9, 89%). Diffusing capacity significantly correlated with the extent of all parenchymal abnormalities in all CVD and IPF, with honeycomb lesion in PSS, and with ground-glass shadow or air-space consolidation in CVD except for PSS (r < -0.7, P < 0.001). In all 15 cases in which corticosteroid therapy was effective, no honeycomb lesions were seen. Collagen vascular disease, except for PSS, had a different pattern of disease than IPF. The morphologic changes seen on HRCT correlated well with pulmonary function in CVD.