Pulmonary function impairment in patients with combined pulmonary fibrosis and emphysema with and without airflow obstruction.

Abstract:

BACKGROUND:The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is a recently described entity associating upper-lobe emphysema and lower-lobe fibrosis. We sought to evaluate differences in pulmonary function between CPFE patients with and without airflow obstruction. SUBJECTS AND METHODS:Thirty-one CPFE patients were divided into two groups according to the presence or absence of irreversible airflow obstruction based on spirometry (forced expiratory volume in 1 second/forced vital capacity <70% following inhalation of a β2-agonist) as follows: CPFE patients with airflow obstruction (CPFE OB(+) group, n=11), and CPFE patients without airflow obstruction (CPFE OB(-) group, n=20). Pulmonary function, including respiratory impedance evaluated using impulse oscillometry and dynamic hyperinflation following metronome-paced incremental hyperventilation, was retrospectively analyzed in comparison with that observed in 49 chronic obstructive pulmonary disease (COPD) patients (n=49). RESULTS:In imaging findings, low-attenuation-area scores on chest high-resolution computed tomography, representing the degree of emphysema, were significantly lower in the CPFE OB(-) group than in the CPFE OB(+) and COPD groups. In contrast, the severity of pulmonary fibrosis was greater in the CPFE OB(-) group than in the CPFE OB(+) group. In pulmonary function, lung hyperinflation was not apparent in the CPFE OB(-) group. Impairment of diffusion capacity was severe in both the CPFE OB(-) and CPFE OB(+) groups. Impulse oscillometry showed that respiratory resistance was not apparent in the CPFE OB(-) group compared with the COPD group, and that easy collapsibility of small airways during expiration of tidal breath was not apparent in the CPFE OB(+) group compared with the COPD group. Dynamic hyperinflation following metronome-paced incremental hyperventilation was significantly greater in the COPD group than in the CPFE OB(-) group, and also tended to be greater in the CPFE OB(+) group than in the CPFE OB(-) group. CONCLUSION:The mechanisms underlying impairment of physiological function may differ among CPFE OB(+) patients, CPFE OB(-) patients, and COPD patients. CPFE is a heterogeneous disease, and may have distinct phenotypes physiologically and radiologically.

authors

Kitaguchi Y,Fujimoto K,Hanaoka M,Honda T,Hotta J,Hirayama J

doi

10.2147/COPD.S65621

subject

Has Abstract

pub_date

2014-07-29 00:00:00

pages

805-11

eissn

1176-9106

issn

1178-2005

pii

copd-9-805

journal_volume

9

pub_type

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