The aim of the present study was to evaluate exercise limiting factors using cardiopulmonary exercise testing (CPET) in patients with idiopathic pulmonary fibrosis (IPF), and to investigate whether these parameters are related to survival after CPET. We evaluated 41 patients with IPF (mean 68.2 years, 27 male) who performed CPET. The exercise capacity in patients with IPF was limited more strongly by gas exchange and/or ventilatory impairments, compared with cardiac impairment. Using univariate analysis, the severity of exercise-induced hypoxemia (EIH) evaluated by deltaPaO2/deltaVO2 (PaO2-slope), oxygen uptake at maximum exercise, oxygen pulse at maximum exercise, ventilatory equivalent for carbon dioxide at maximum exercise and age were significantly related to the survival rate. Interestingly, the PaO2-slope was most closely correlated with the survival rate using multiple analysis with a stepwise evaluation. Nevertheless, PaO2 at rest and at maximum exercise were not factors influencing survival. In patients with IPF, CPET can simultaneously evaluate the ability of both the cardiovascular and respiratory systems, and should be available so that parameters can be derived to make the necessary prognostic estimations, with the most useful parameter being the degree of EIH as represented by the PaO2-slope.