TY  - JOUR
A1  - Gredic, Marija
A1  - Karnati, Srikanth
A1  - Ruppert, Clemens
A1  - Guenther, Andreas
A1  - Avdeev, Sergey N.
A1  - Kosanovic, Djuro
T1  - Combined pulmonary fibrosis and emphysema: when Scylla and Charybdis ally
JF  - Cells
N2  - Combined pulmonary fibrosis and emphysema (CPFE) is a recently recognized syndrome that, as its name indicates, involves the existence of both interstitial lung fibrosis and emphysema in one individual, and is often accompanied by pulmonary hypertension. This debilitating, progressive condition is most often encountered in males with an extensive smoking history, and is presented by dyspnea, preserved lung volumes, and contrastingly impaired gas exchange capacity. The diagnosis of the disease is based on computed tomography imaging, demonstrating the coexistence of emphysema and interstitial fibrosis in the lungs, which might be of various types and extents, in different areas of the lung and several relative positions to each other. CPFE bears high mortality and to date, specific and efficient treatment options do not exist. In this review, we will summarize current knowledge about the clinical attributes and manifestations of CPFE. Moreover, we will focus on pathophysiological and pathohistological lung phenomena and suspected etiological factors of this disease. Finally, since there is a paucity of preclinical research performed for this particular lung pathology, we will review existing animal studies and provide suggestions for the development of additional in vivo models of CPFE syndrome.
KW  - CPFE
KW  - lung fibrosis
KW  - emphysema
KW  - animal models
Y1  - 2023
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-313571
SN  - 2073-4409
VL  - 12
IS  - 9
ER  -