Can a Person Have Both ILD and Emphysema Simultaneously?
Yes, a person can absolutely have both interstitial lung disease (ILD) and emphysema at the same time—this is a well-recognized clinical syndrome called Combined Pulmonary Fibrosis and Emphysema (CPFE). 1
Understanding CPFE as a Distinct Syndrome
CPFE is formally recognized as a syndrome by the American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association, characterized by the coexistence of upper-lobe emphysema and lower-lobe pulmonary fibrosis. 2 This is not merely coincidental overlap but represents a distinct clinical entity with:
- Shared pathogenetic pathways between the two disease processes 2
- Unique clustering patterns that distinguish it from either disease alone 2
- Specific prognostic implications including increased mortality risk 2
Epidemiology and Prevalence
CPFE is surprisingly common among patients with fibrotic ILD:
- Among patients with idiopathic pulmonary fibrosis (IPF), 39% have concurrent emphysema (CPFE-IPF) 3
- Among patients with other fibrotic ILDs, 36.3% have concurrent emphysema (CPFE-fILD) 3
- Prevalence ranges from 71-100 per 100,000 in veteran populations 3
- Annual incidence ranges from 16-39 per 100,000 3
Clinical Presentation: The Diagnostic Challenge
CPFE presents a unique diagnostic challenge because emphysema and fibrosis counterbalance each other on standard testing, creating deceivingly normal-appearing results despite severe disease. 4
Key Clinical Features:
- Severe exertional dyspnea disproportionate to spirometry findings 2
- Predominantly male patients with significant smoking history 2, 3
- Preserved or near-normal lung volumes on spirometry (emphysema increases volumes, fibrosis decreases them) 1, 2
- Severely impaired diffusing capacity (DLCO) despite normal spirometry 2, 3
- Profound exertional hypoxemia 2
- Lower BMI compared to ILD alone 3
Radiologic Recognition
High-resolution CT (HRCT) is essential for diagnosis and will show the characteristic pattern of upper-lobe emphysema with lower-lobe fibrosis. 1
HRCT Patterns in Smokers with ILD:
Multiple patterns may coexist including:
- Langerhans cell histiocytosis 1
- Respiratory bronchiolitis 1
- Desquamative interstitial pneumonia 1
- Pulmonary fibrosis (UIP or NSIP patterns) 1
- Emphysema 1
Critical Diagnostic Pitfall
The most dangerous pitfall is attributing preserved lung volumes to mild disease severity when CPFE is actually present. 5
- You must actively search for emphysema on every HRCT performed for ILD diagnosis 5
- Preserved FVC in the setting of severe dyspnea and low DLCO should raise immediate suspicion for CPFE 5
- Monitoring FVC alone does not allow accurate assessment of disease progression in CPFE 5
Prognostic Implications and Complications
CPFE carries a dismal prognosis with significantly increased risk of life-threatening complications compared to either disease alone. 2
Major Complications:
- Pulmonary hypertension is particularly frequent and represents the main predictor of mortality 5, 2
- Increased risk of lung cancer 6, 2
- Higher risk of acute exacerbations 7
- Increased overall mortality in unadjusted analyses 3
After adjustment for age, sex, and BMI, CPFE mortality is similar to ILD without emphysema (HR 1.13 for CPFE-IPF vs IPF alone, 95% CI 0.96-1.33). 3 However, the absolute mortality burden remains high due to the severe baseline disease.
Management Approach
Essential Initial Steps:
- Immediate smoking cessation is mandatory 5
- Screen for pulmonary hypertension given the high prevalence and prognostic significance 5
- Assess for airflow obstruction and prescribe inhaled bronchodilators if present 5
- Evaluate oxygen requirements, as these patients often need supplemental oxygen 5
Treatment Considerations:
No specific data support unique pharmacologic management of CPFE versus ILD alone. 5 Treatment decisions must be individualized considering:
- Antifibrotic therapy (pirfenidone or nintedanib) should be considered based on the fibrotic component 5
- The absence of evidence for benefit in CPFE specifically 5
- The difficulty evaluating disease evolution due to minimal FVC changes 5
- Potential side effects of therapy 5
Monitoring Strategy:
- FVC and DLCO monitoring does not allow accurate prognosis assessment 5
- Serial HRCT imaging is more useful for tracking progression 5
- Regular assessment for pulmonary hypertension development 5
- Long-term oxygen therapy is frequently indicated 5
Recognition Across ILD Subtypes
CPFE is not limited to IPF—it occurs across a wide variety of interstitial lung diseases including:
This broad occurrence emphasizes that clinicians must actively look for emphysema in all patients with fibrotic ILD, regardless of the underlying ILD subtype.