The natural history of the pulmonary involvement in systemic sclerosis is not well studied. Reported here are the serial measurements of pulmonary function in a well defined population of patients with systemic sclerosis followed over a mean of 63 months. The mean rate of loss of vital capacity (0.10 liters per year) for the 38 patients serially studied was more than three times the expected rate of loss for a normal population. The mean rate of loss of diffusing capacity (0.33 ml/min/mm Hg per year) for the 27 patients serially studied was similar to that for a normal population. The percent forced vital capacity expired in the first second increased 0.53 percent per year consistent with a progressive restrictive ventilatory defect. Smokers tended to lose vital capacity at a slightly greater rate than nonsmokers (p = 0.069). Individual variability in the course of pulmonary function was observed. Although the overall trend in our population was towards a slowly progressive restrictive ventilatory defect, pulmonary function in the nonsmokers did not change at rates different from those in a nonsmoking reference population.