Idiopathic interstitial pulmonary fibrosis with hypercapnia

A 38-year-old patient with effort dyspnea, somnolence, cianosis and cor pulmonale is presented. Chest roentgenograms and lung function studies suggested the diagnosis of pulmonary fibrosis. The patient showed also severe hypercapnia with normal resting ventilation and ventilatory response to exercise lower than usual for this condition. Autopsy confirmed the clinical diagnosis. This subject may belong to the growing group of patients where CO2 retention is not explained by their pulmonary pathology.