Progressive systemic sclerosis (PSS) is a chronic multisystemic disease characterized by vascular changes, lung fibrosis and skin conditions. The lung is one of the most commonly involved organs in the patients suffering from this disease. Lung changes, along with heart involvement, represent one of the major causes of death in PSS patients. CT and high-resolution computed tomography (HRCT) scans and chest radiographs were obtained in 26 patients with PSS. The evaluation of chest films included the assessment of an average profusion score. The HRCT evaluation included pattern, extent and distribution of the disease. HRCT scans showed thickened septal lines in all patients and parallel subpleural lines in 6 patients (23%), while ground-glass opacification was seen in 2 cases (7.6%). Honeycomb pattern was observed in 8 patients (31%). Chest films showed definite interstitial opacification patterns in 8 cases only; 6 radiographs were equivocal and 12 (46%) normal. Five patients were followed 6 months to 3 years: 2 of them exhibited parallel worsening of skin conditions and of CT patterns, while X-ray findings in the chest were unchanged. In 2 cases skin and lung involvement were different (skin worsening and unchanged lung patterns, or viceversa). Finally, in 1 patient, the presence of patchy areas of air-space consolidation without air cysts on second-look was more consistent with bronchiolitis obliterans organizing pneumonia.