open access
IPF and CPFE — the two different entities or two different presentations of the same disease?
open access
Abstract
In this article the co-existence of pulmonary emphysema with lung fibrosis of typical pattern and distribution for usual interstitial
pneumonia (UIP) was compared with idiopathic pulmonary fibrosis (IPF) alone. Author discusses the etiopathogenesis of these
diseases, differences in signaling pathways and the role of senescent cells. Moreover, clinical course, pulmonary function tests
as well as main complications are reviewed. However, the lack of well-established diagnostic criteria for CPFE along with mainly
retrospective character of the studies make current knowledge about this entity rather deficient.
Abstract
In this article the co-existence of pulmonary emphysema with lung fibrosis of typical pattern and distribution for usual interstitial
pneumonia (UIP) was compared with idiopathic pulmonary fibrosis (IPF) alone. Author discusses the etiopathogenesis of these
diseases, differences in signaling pathways and the role of senescent cells. Moreover, clinical course, pulmonary function tests
as well as main complications are reviewed. However, the lack of well-established diagnostic criteria for CPFE along with mainly
retrospective character of the studies make current knowledge about this entity rather deficient.
Keywords
idiopathic pulmonary fibrosis, IPF, combined pulmonary fibrosis and emphysema, CPFE
About this articleTitle
IPF and CPFE — the two different entities or two different presentations of the same disease?
Journal
Advances in Respiratory Medicine
Issue
Pages
23-26
Published online
2017-12-29
DOI
10.5603/ARM.a2017.0049
Bibliographic record
Adv Respir Med 2018;86(1):23-26.
Keywords
idiopathic pulmonary fibrosis
IPF
combined pulmonary fibrosis and emphysema
CPFE
Authors
Sylwia Kwiatkowska