From the Guidelines
Combined pulmonary fibrosis and emphysema (CPFE) is considered a form of interstitial lung disease (ILD) due to its fibrotic component, which involves inflammation and scarring of the lung interstitium. CPFE is a distinct clinical syndrome characterized by the coexistence of upper lobe emphysema and lower lobe pulmonary fibrosis in the same patient. The definition of ILD provided by the American Thoracic Society, as stated in the 2012 guideline 1, includes acute and chronic bilateral parenchymal infiltrative lung diseases with variable degrees of tissue inflammation and fibrosis, which applies to CPFE.
Key characteristics of CPFE include:
- Coexistence of emphysema and pulmonary fibrosis
- Typically affects older male smokers
- Presents with severe dyspnea and preserved lung volumes despite significantly reduced diffusion capacity
- High prevalence of pulmonary hypertension
Management of CPFE focuses on:
- Smoking cessation
- Oxygen therapy when indicated
- Treatment of complications
- Consideration of antifibrotic medications like pirfenidone or nintedanib for the fibrotic component, although their efficacy in CPFE is less established than in idiopathic pulmonary fibrosis alone 1.
The dual pathology of CPFE makes it particularly challenging to treat, as medications beneficial for fibrosis might not address the emphysematous changes, and the condition generally carries a worse prognosis than either disease alone.
From the Research
Definition and Classification of Combined Pulmonary Fibrosis and Emphysema (CPFE)
- CPFE is considered a syndrome characterized by the coexistence of emphysema and fibrotic interstitial lung disease (ILD) 2, 3, 4, 5, 6.
- The syndrome is often associated with a history of smoking, severe dyspnea, relatively preserved airflow rates and lung volumes on spirometry, severely impaired diffusing capacity, exertional hypoxemia, frequent pulmonary hypertension, and a dismal prognosis 2.
Relationship between CPFE and Interstitial Lung Disease (ILD)
- CPFE is characterized by the presence of both emphysema and fibrotic ILD, with varying features of ILD and emphysema 2, 3, 4, 5.
- The most common histological pattern of ILD described in CPFE is usual interstitial pneumonia (UIP), but other patterns such as desquamative interstitial pneumonia and unclassifiable ILD have also been reported 3, 5.
- CPFE can be considered a form of ILD, as it involves the presence of fibrotic lung disease, but it is a distinct entity with its own clinical and radiological characteristics 2, 5.
Clinical and Radiological Characteristics of CPFE
- Patients with CPFE often have preserved lung volumes, but severely impaired diffusing capacity and exertional hypoxemia 2, 4.
- The radiological pattern of CPFE can be heterogeneous, with varying degrees of emphysema and fibrosis, and can include features such as honeycombing and ground glass opacities 3, 5.
- The clinical and radiological characteristics of CPFE can vary depending on the underlying ILD diagnosis and the extent of emphysema and fibrosis 4, 5.