Syndrome of combined pulmonary fibrosis and emphysema – CPFE syndrome

Česká verzia

Authors: M. Vašáková ¹; P. Žáčková ¹; R. Matěj ²
Authors place of work: 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 the journal: Prakt. Lék. 2009; 89(6): 287-289
Category: Reviews

Summary

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.

Zdroje

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