Concomitant upper-lobe bullous emphysema, lower-lobe interstitial fibrosis and pulmonary hypertension in heavy smokers: report of eight cases and review of the literature

Abstract

Our findings are in line with emerging evidence that the spectrum of interstitial damage caused by smoke includes not only Langerhans cell hystiocytosis, respiratory bronchiolitis or desquamative interstitial pneumonia but also advanced usual interstitial pneumonitis as well. We believe that in some patients smoking plays a destructive role by a variety of mechanisms and can cause emphysema, lung fibrosis as well as pulmonary vasculopathy and hypertension. Future studies are needed to define the genetics and pathophysiology of this uncommonly reported clinical syndrome.

The impact of emphysema in pulmonary fibrosis

“…CPFE is most often observed in males (mean age of 65 years) who are tobacco smokers or ex-smokers of .40 pack-years [9,10]. CPFE has a poor prognosis, with a 5-year survival of 55% [4].…”

Combined Pulmonary Fibrosis and Emphysema

“…Since its initial description, CPFE has eventually been reported in a few case series and case reports. [2][3][4][5][6][7] As first described by Wiggins et al and further reinforced in later case series, CPFE seems to occur mostly in males past their sixth decade. The underlying cause for CPFE has not been established.…”

Concomitant Systemic Sclerosis and Sarcoidosis with Combined Pulmonary Fibrosis and Emphysema

“…CPFE is reported to have a strong correlation with cigarette smoking, and most patients present with approximately 40-pack-year histories or more (1,5). A retrospective study of 61 patients with CPFE revealed elevated ANA levels in a third of the patients, though none of them had overt CTD when diagnosed with CPFE (1).…”