open access

Ahead of Print
REVIEWS
Published online: 2017-12-29
Submitted: 2017-11-02
Accepted: 2017-12-29
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Idiopathic pulmonary fibrosis (IPF) and combined pulmonary fibrosis with emphysema (CPFE) ― different entities or different presentations of the same disease?

Sylwia Kwiatkowska
DOI: 10.5603/ARM.a2017.0049

open access

Ahead of Print
REVIEWS
Published online: 2017-12-29
Submitted: 2017-11-02
Accepted: 2017-12-29

Abstract

CPFE and IPF seem to be quite similar diseases. In this article the co-existence of pulmonary emphysema with typical distribution and UIP pattern is compared with 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 data mainly from retrospective studies make that current knowledge about this entity is rather scarce..    

Abstract

CPFE and IPF seem to be quite similar diseases. In this article the co-existence of pulmonary emphysema with typical distribution and UIP pattern is compared with 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 data mainly from retrospective studies make that current knowledge about this entity is rather scarce..    
Get Citation

Keywords

idiopathic pulmonary fibrosis, IPF, combined pulmonary fibrosis and emphysema, CPFE

About this article
Title

Idiopathic pulmonary fibrosis (IPF) and combined pulmonary fibrosis with emphysema (CPFE) ― different entities or different presentations of the same disease?

Journal

Advances in Respiratory Medicine

Issue

Ahead of Print

Published online

2017-12-29

DOI

10.5603/ARM.a2017.0049

Keywords

idiopathic pulmonary fibrosis
IPF
combined pulmonary fibrosis and emphysema
CPFE

Authors

Sylwia Kwiatkowska

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