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.
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| http://dx.doi.org/10.5603/ARM.a2017.0049 | DOI Listing |
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Combined pulmonary fibrosis and emphysema and lung transplantation: current evidence and future directions.
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: Pulmonary hypertension (PH) often accompanies chronic lung diseases. Several chronic lung diseases with PH portends unfavorable outcomes. We investigated which variables in this cohort of patients with chronic lung disease and PH predicts mortality.
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Article Synopsis
- A study on combined pulmonary fibrosis and emphysema (CPFE) explored how these conditions impact mortality and lung function decline compared to patients with just idiopathic pulmonary fibrosis (IPF).
- CPFE patients with more than 10% emphysema had a history of stronger smoking habits and experienced more pronounced declines in lung function, particularly in diffusing capacity, which correlated better with mortality than overall lung capacity measures.
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Department of Pulmonology, I.M. Sechenov First Moscow State Medical University (Sechenov University), 119991 Moscow, Russia.
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.
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