Abstract
Pulmonary function tests in patients with idiopathic pulmonary fibrosis characteristically show a restrictive pattern including small lung volumes and increased expiratory flow rates resulting from a reduction in pulmonary compliance due to diffuse fibrosis. Conversely, an obstructive pattern with hyperinflation results in emphysema by loss of elastic recoil, expiratory collapse of the peripheral airways and air trapping. When the diseases coexist, pulmonary volumes are compensated, and a smaller than expected reduction or even normal lung volumes can be found. The present report describes 10 patients with progressive breathlessness, three of whom experienced severe limitation in their quality of life. All patients showed lung interstitial involvement and emphysema on computed tomography scan of the chest. The 10 patients showed normal spirometry and lung volumes with severe compromise of gas exchange. Normal lung volumes do not exclude diagnosis of idiopathic pulmonary fibrosis in patients with concomitant emphysema. The relatively preserved lung volumes may underestimate the severity of idiopathic pulmonary fibrosis and attenuate its effects on lung function parameters.
Highlights
Pulmonary function tests in patients with idiopathic pulmonary fibrosis characteristically show a restrictive pattern including small lung volumes and increased expiratory flow rates resulting from a reduction in pulmonary compliance due to diffuse fibrosis
The patient was offered the option of referral to a transplant centre for assessment for heart-lung transplant, but he declined. He passed away from respiratory failure four months after initial assessment. This individual was one of a series of 10 patients who we reviewed at the Royal University Hospital who had a combination of emphysema and interstitial lung disease, one of whom has been previously reported [1]
An isolated reduction in DLCO is extremely rare (
Summary
A49-year-old truck driver was referred to the respirology outpatient clinic of the Royal University Hospital (Saskatoon, Saskatchewan) for evaluation of an abnormal immigration chest radiograph. The patient was offered the option of referral to a transplant centre for assessment for heart-lung transplant, but he declined He passed away from respiratory failure four months after initial assessment. This individual was one of a series of 10 patients who we reviewed at the Royal University Hospital who had a combination of emphysema and interstitial lung disease, one of whom (patient 8 [Table 1]) has been previously reported [1]. None of these patients demonstrated improvement of their interstitial lung disease while taking corticosteroids Five of these patients have since passed away from progressive respiratory failure, with a mean survival of 23 months from first assessment. Two of these patients had minimal dyspnea when first assessed (patients 5 and 7)
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