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.
Highlights
At the first glimpse the two entities are very similar
Cenrtilobular and/or paraseptal emphysema or bullous changes in the upper lobes are characteristic of Combined pulmonary fibrosis and emphysema (CPFE)
Emphysema can be present in Idiopathic pulmonary fibrosis (IPF) patients as reticulation admixed emphysema and may mimic honeycombing [2, 3]
Summary
Idiopathic pulmonary fibrosis (IPF) is a progressive disease with fibrosis of the lung alveoli and interstitium of unknown etiology, but poor prognosis (median survival 3–4 years). Emphysema concomitant with interstitial lung disease is specific for IPF pattern [4]. They proved that CPFE is a distinct entity, different from IPF as well as from emphysema, with distinct prognosis and typical comorbidities (i.e. pulmonary hypertension).
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