Syndrome of combined pulmonary fibrosis and emphysema – CPFE syndrome
Authors:
M. Vašáková 1; P. Žáčková 1; R. Matěj 2
Authors‘ workplace:
Pneumologická klinika 1. LF UK
Přednosta: prof. MUDr. Jiří Homolka, DrSc.
1; Odddělení patologie a molekulární mediciny
Primář: MUDr. František Koukolík, DrSc.
Fakultní Thomayerova nemocnice s poliklinikou, Praha
2
Published in:
Prakt. Lék. 2009; 89(6): 287-289
Category:
Reviews
Overview
Pulmonary fibrosis and emphysema have been considered as two entirely different groups of diseases. In spite of this, combined pulmonary fibrosis and emphysema (CPFE) syndrome, where the two pathologic phenotypes (fibrosis and emphysema) are described in one patient, has been repeatedly described in past 20 years. The clinical, functional and morphologic phenotype corresponds to both diseases in combination. Probable common pathogenetic feature of emphysema and fibrosis is transforming growth factor beta (TGF-β), the cytokine that plays a crucial role in process of healing and fibrogenesis. TGF-β is likely to influence the mechanism of epithelial- mesenchymal transdifferentiation (EMT) and even epithelial- mesenchymal crosstalk (EMC), that are the main pathogenetic mechanisms of diffuse interstitial lung diseases, both fibrosis and emphysema. From the clinical point of view, few forms of CPFE exist, according to emphysema and fibrosis ratio and mutual position. CPFE patient’s prognosis is unfavourable despite treatment, but probably better then in isolated idiopathic pulmonary fibrosis.
Key words:
pulmonary fibrosis, emphysema, pathogenesis, clinical presentation.
Sources
1. Cottin, V., Nunes, H., Brillet, P-Y. et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognized entity. Eur. Respir. J. 2005, 26, p. 586-593.
2. Gauldie, J., Kolb, M., Kjetil, A. et al. Smad 3 signaling involved in pulmonary fibrosis and emphysema. Proc. Am. Thorac. Soc. 2006, 3, p. 696-670.
3. Lucattelli, M., Bartalesi, B., Cavarra, E. et al. Is neutrophil elastase the missing link between emphysema and fibrosis? Evidence from two mouse models. Respir. Res. 2005; 6, p. 83-96.
4. Lundblad, L.K.A., Thompson-Figueroa, J., Leclair, T. et al. Tumor necrosis factor alpha overexpression in lung disease. A single cause behind a complex phenotype. Am. J. Respir. Crit. Care Med. 2005, 171, p.1363-1370.
5. Lungarella, G., Cavarra, E., Lucattelli, M. et al. The dual role of neutrophil elastase in lung destruction and repair. Int. J. Biochem. Cell Biol. 2008, 40(6-7), p. 1287-1296.
6. Vašáková, M. Idiopatická plicní fibróza - kryptogenní fibrotizující alveolitida. Postgrad. med. 2009, 11(2), s. 133- 137.
Labels
General practitioner for children and adolescents General practitioner for adultsArticle was published in
General Practitioner
2009 Issue 6
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