Combined pulmonary fibrosis and emphysema syndrome (CFPE)

Introduction: CPFE is a clinic entity which consists in the coexistence of emphysema of the upper lobes and pulmonary fibrosis of the lower lobes. Method: Retrospective descriptive study of cases diagnosed of CPFE between 2007 and 2012. Results: 44 patients, all of them men, with an average age of 69 years. All were current or ex smokers. 50% had a UIP (usual interstitial pneumonia) pattern at the HRTC, 11.4% possible UIP pattern, and 38.6 % inconsistent UIP pattern. All the patients had emphysema, above all paraseptal and centrilobular. PAH was present in 10 patients. The final diagnosis was: 18 IPF, 1 possible IPF,1 NSIP, 3 possible NSIP, 6 fibrosis associated connective tissue diseases, 1 asbestosis, 1 hypersensitivity pneumonitis, 1 drug associated fibrosis, and 12 non classificable interstitial pneumonias. During follow-up 8 patients died, 6 were diagnosed of lung cancer View this table: Results Conclusions: All of the patients from this study are men with smoking history. These patients have, lung volumes preserved with a severe impairment of gas exchange. The high prevalence of PAH and its important role in the prognosis justify echocardiography. As these patients might have a high prevalence of lung cancer, a close follow-up would be advisable.