open access

Vol 86, No 1 (2018)
REVIEWS
Published online: 2017-12-29
Submitted: 2017-11-02
Accepted: 2017-12-29
Get Citation

IPF and CPFE — the two different entities or two different presentations of the same disease?

Sylwia Kwiatkowska
DOI: 10.5603/ARM.a2017.0049
·
Adv Respir Med 2018;86(1):23-26.

open access

Vol 86, No 1 (2018)
REVIEWS
Published online: 2017-12-29
Submitted: 2017-11-02
Accepted: 2017-12-29

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.

Get Citation

Keywords

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

About this article
Title

IPF and CPFE — the two different entities or two different presentations of the same disease?

Journal

Advances in Respiratory Medicine

Issue

Vol 86, No 1 (2018)

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

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