RT Journal Article
SR Electronic
T1 Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 586
OP 593
DO 10.1183/09031936.05.00021005
VO 26
IS 4
A1 V. Cottin
A1 H. Nunes
A1 P-Y. Brillet
A1 P. Delaval
A1 G. Devouassoux
A1 I. Tillie-Leblond
A1 D. Israel-Biet
A1 I. Court-Fortune
A1 D. Valeyre
A1 J-F. Cordier
A1 the Groupe d'Etude et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM“O”P)
YR 2005
UL http://erj.ersjournals.com/content/26/4/586.abstract
AB 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.