Management of Pulmonary Emphysema
Smoking cessation using combination pharmacotherapy (varenicline or bupropion PLUS nicotine replacement therapy) alongside intensive behavioral counseling is the absolute first priority and the only intervention proven to slow disease progression, reduce mortality, and improve long-term outcomes in emphysema. 1, 2
Immediate Priority: Smoking Cessation
- Implement high-intensity cessation immediately with combination pharmacotherapy: nicotine replacement therapy (patch PLUS rapid-acting form like gum or lozenge) PLUS either varenicline or bupropion SR 1, 3
- Provide intensive behavioral support including individual counseling sessions, telephone follow-up contacts, and small-group sessions 3
- Advise abrupt cessation rather than gradual reduction, as gradual withdrawal rarely achieves complete cessation 1, 2
- Smoking cessation is the only treatment that modifies the natural decline in lung function—when smokers quit, their subsequent lung function decline returns to rates similar to healthy non-smokers 2
- This approach reduces exacerbations (0.38 vs 0.60 per patient) and hospital days (0.39 vs 1.00 per patient) compared to less intensive strategies 3
- Expect multiple quit attempts as approximately one-third succeed with support; heavy smokers with multiple previous attempts require even more intensive support 1, 3
Pharmacologic Bronchodilator Therapy
- Initiate long-acting bronchodilator therapy immediately to reduce symptoms, prevent exacerbations, and improve exercise tolerance 2
- Start with either a long-acting β2-agonist (LABA) or long-acting anticholinergic (LAMA such as tiotropium) 1, 2
- Combination LABA/LAMA therapy (such as tiotropium/olodaterol) provides superior bronchodilation compared to monotherapy, with improvements in FEV1 maintained over 52 weeks 4
- Teach proper inhaler technique at first prescription and verify at every visit, as older age, use of multiple devices, and lack of previous education predict poor technique 5, 1
- Continue bronchodilator therapy even if spirometric improvement is modest, as symptom relief and functional capacity can improve regardless of FEV1 changes 1, 3
Inhaled Corticosteroids: Selective Use Only
- Consider adding inhaled corticosteroids (ICS) to LABA therapy only if FEV1 decline is rapid (>50 mL/year) or for patients with frequent exacerbations (≥2 per year) 1
- Do not use ICS as monotherapy 1
- In patients with severe COPD (FEV1 <50% predicted) associated with chronic bronchitis and ≥2 exacerbations per year, roflumilast 500 mcg once daily reduces exacerbation rates by 15-18% compared to placebo 6
- Monitor for pneumonia risk with ICS use, as different formulations confer varying risk 5
Vaccinations to Reduce Mortality
- Administer influenza vaccine annually to reduce serious illness, death, risk of ischemic heart disease, and total exacerbations 2
- Provide pneumococcal vaccines (PCV13 and PPSV23) for all patients 65 years and older; younger patients with emphysema should also receive them 2
Pulmonary Rehabilitation
- Refer to pulmonary rehabilitation program to improve symptoms, quality of life, and physical and emotional participation in everyday activities, with benefits occurring regardless of disease severity 5, 2
- Pulmonary rehabilitation reduces hospitalizations, improves dyspnea, and enhances exercise capacity 5
- Initiate rehabilitation more than 4 weeks after hospitalization for exacerbation, as starting before hospital discharge may compromise survival 5
Assessment for Severe Disease Requiring Life-Prolonging Interventions
- Perform spirometry to assess severity and check arterial blood gas or pulse oximetry at rest to evaluate for hypoxemia 2
- Measure FEV1 as the primary metric for severity staging and prognosis—it predicts mortality better than FEV1/FVC ratio 1
- Document FEV1 % predicted to classify disease severity: mild (≥80%), moderate (50-79%), severe (30-49%), or very severe (<30%) 1
- Obtain chest radiograph to exclude lung cancer, pneumonia, pneumothorax, and assess for cor pulmonale (right descending pulmonary artery >16mm suggests pulmonary hypertension) 1
- Check arterial blood gases if FEV1 <50% predicted or clinical signs of respiratory failure or cor pulmonale 1
- Measure alpha-1 antitrypsin level if emphysema is suspected, particularly in younger patients or those with basilar-predominant disease 1
Long-Term Oxygen Therapy (LTOT)
- Prescribe LTOT (>15 hours/day) if PaO2 ≤55 mmHg (7.3 kPa) or PaO2 56-59 mmHg with evidence of cor pulmonale or polycythemia 1
- LTOT is the only treatment besides smoking cessation known to improve prognosis in patients with severe COPD and hypoxemia 2
- Target oxygen saturation of 88-92% if respiratory acidosis develops 2
- Long-term oxygen therapy does not benefit patients with stable COPD and only resting or exercise-induced moderate arterial oxygen desaturation 5
Management of Acute Exacerbations
- Initiate empirical antibiotics for 7-14 days if sputum becomes purulent (amoxicillin, tetracycline derivatives, or amoxicillin/clavulanic acid based on local resistance patterns) 1, 3
- Common pathogens include Streptococcus pneumoniae, Haemophilus influenzae, and Moraxella catarrhalis 3
- Increase bronchodilator frequency/dose and administer a short course of systemic corticosteroids for acute exacerbations 1
Screen for Comorbidities That Impact Mortality
- Assess for cardiovascular disease aggressively, as approximately 26% of deaths in moderate to severe COPD are cardiovascular 2
- Screen for lung cancer, osteoporosis, depression, and anxiety 1
- Monitor bone mineral density in patients on long-term ICS 1
- Treat depression and anxiety with selective serotonin reuptake inhibitors, as tricyclic antidepressants may be poorly tolerated in those with chronic sputum production 5
Monitoring and Follow-Up
- Perform spirometry at every follow-up visit to monitor disease progression 1, 2
- Schedule follow-up within 2-4 weeks after exacerbation to assess response to treatment 1, 2
- Monitor arterial blood gases if abnormal at initial assessment 1
- Check medication adherence, symptom relief, inhaler technique, smoking status, FEV1, and vital capacity at each visit 1
Critical Pitfalls to Avoid
- Do not rely on physical examination alone to assess COPD severity—absence of wheezing does not exclude significant disease 3
- Do not discontinue oxygen abruptly if respiratory acidosis develops; instead step down to 28-35% Venturi mask or 1-2 L/min nasal cannula targeting SpO2 88-92% 3
- Do not recommend gradual smoking reduction as the primary strategy—it rarely achieves complete cessation 1, 3
- Do not use ICS as monotherapy 1
Advanced Interventions for Selected Patients
- Consider lung volume reduction surgery (LVRS) for selected patients with emphysema, as it has an impact on mortality in appropriate candidates 7
- Noninvasive ventilation during severe exacerbations has an impact on mortality 7
- Lung transplantation improves patient-centered outcomes such as health-related quality of life, dyspnea, and exercise capacity 7
Alpha-1 Antitrypsin Deficiency-Specific Management
- For patients with severe alpha-1 antitrypsin deficiency and emphysema, intravenous augmentation therapy with purified human AAT concentrate (60 mg/kg weekly) increases lung levels of AAT to 60-70% of normal 5
- Observational studies suggest reduction in spirometric progression with augmentation therapy, and CT scan studies provide evidence for preserving lung tissue compared with placebo 5