d’Etude et de Recherche sur les Maladies ‘‘Orphelines’ ’ Pulmonaires

ABSTRACT: The syndrome resulting from combined pulmonary fibrosis and emphysema has not been comprehensively described. The current authors conducted a retrospective study of 61 patients with both emphysema of the upper zones and diffuse parenchymal lung disease with fibrosis of the lower zones of the lungs on chest computed tomography. Patients (all smokers) included 60 males and one female, with a mean age of 65 yrs. Dyspnoea on exertion was present in all patients. Basal crackles were found in 87 % and finger clubbing in 43%. Pulmonary function tests were as follows (mean¡SD): total lung capacity 88%¡17, forced vital capacity (FVC) 88%¡18, forced expiratory volume in one second (FEV1) 80%¡21 (% predicted), FEV1/FVC 69%¡13, carbon monoxide diffusion capacity of the lung 37%¡16 (% predicted), carbon monoxide transfer coefficient 46%¡19. Pulmonary hypertension was present in 47 % of patients at diagnosis, and 55 % during follow-up. Patients were followed for a mean of 2.1¡2.8 yrs from diagnosis. Survival was 87.5 % at 2 yrs and 54.6 % at 5 yrs, with a median of 6.1 yrs. The presence of pulmonary hypertension at diagnosis was a critical determinant of prognosis. The authors hereby individualise the computer tomography-defined syndrome of combined pulmonary fibrosis and emphysema characterised by subnormal spirometry, severe impairment of gas exchange, high prevalence of pulmonary hypertension, and poor survival