Table 1 Physiology in CPFE. Clinical implications

• The finding of preserved or slightly reduced lung volumes does not rule out the presence of fibrosis

• CPFE patients tend to exhibit a delay in the reduction of FVC and DLco.

• Decline in FEV1 is the strongest predictor of mortality in CPFE patients and its prognostic validity increases with increasing severity of emphysema in a dose-dependent fashion

• A decline in DLco should be viewed cautiously, as it could be the result of development/progression of pulmonary hypertension which is correlated with a bad prognosis and may alert the physician towards early referral for lung transplantation.