From the Guidelines
Yes, Combined Pulmonary Fibrosis and Emphysema (CPFE) is a recognized term in pulmonary medicine. CPFE is a clinical syndrome characterized by the coexistence of upper lobe emphysema and lower lobe pulmonary fibrosis in the same patient, as noted in a study published in the European Respiratory Review 1. This condition is significant because patients with CPFE have preserved lung respiratory volumes, decreased carbon monoxide transfer, and higher oxygen requirements compared to those with idiopathic pulmonary fibrosis (IPF) alone. The diagnosis of CPFE is crucial to avoid misattributing the preservation of pulmonary volumes to a milder disease severity. Key characteristics of CPFE include:
- Preserved lung volumes despite reduced diffusion capacity
- High prevalence of pulmonary hypertension, which is a main predictor of mortality
- Potential for worse disease evolution compared to IPF alone
- Importance of long-term oxygen therapy in management
Management of CPFE is complex due to the potential for therapies beneficial for emphysema to worsen fibrosis and vice versa. Therefore, treatment approaches typically focus on:
- Smoking cessation
- Oxygen therapy when indicated
- Pulmonary rehabilitation
- Treatment of complications, such as pulmonary hypertension
The prognosis for CPFE is generally worse than for either pulmonary fibrosis or emphysema alone, with a median survival ranging from 3 to 6 years after diagnosis, although specific survival data was not provided in the referenced study 1.
From the Research
Definition and Recognition of CPFE
- Combined Pulmonary Fibrosis and Emphysema (CPFE) is a recognized term in pulmonary medicine, referring to the coexistence of pulmonary fibrosis and emphysema in the lungs 2, 3, 4, 5, 6.
- CPFE is characterized by the presence of upper lobe emphysema and lower lobe fibrosis, with patients experiencing severe dyspnea and impaired gas exchange despite preserved lung volumes 3, 5.
Clinical Features and Outcomes
- Patients with CPFE are typically heavy smokers or former smokers, with a history of severe breathlessness and low DLCO 2, 3.
- CPFE is associated with a high risk of developing lung cancer and pulmonary arterial hypertension, which can lead to a poor prognosis 3, 5.
- The mortality rate for CPFE is significant, with a median survival range of 2.1 to 8.5 years 5.
Diagnosis and Research
- The diagnosis of CPFE is based on high-resolution computed tomography (HRCT) imaging, which shows the coexistence of emphysema and pulmonary fibrosis in the lungs 2, 5.
- Research on CPFE is ongoing, with a need for more studies to understand the pathogenesis, clinical characteristics, and prognostic factors of the condition 3, 4.
- A research definition and classification criteria for CPFE have been proposed, which includes a comprehensive description of radiologic and pathological patterns 4.