Management of Restrictive Lung Disease
Pharmacologic Therapy
- For patients with restrictive lung disease associated with chronic bronchitis, the American Thoracic Society recommends considering roflumilast in patients with forced expiratory volume in one second (FEV1) <50% predicted 1, 2
- Low-dose long-acting oral and parenteral opioids may be considered for treating dyspnea in patients with severe disease, as suggested by the American College of Chest Physicians 1
- Medications should be selected based on symptom severity, risk of exacerbations, and comorbidities, according to the American Thoracic Society 3
Oxygen Therapy
- Long-term oxygen therapy is indicated for stable patients with low oxygen levels (PaO2 ≤55 mmHg or SaO2 ≤88%), with or without high carbon dioxide levels, confirmed twice over 3 weeks, as recommended by the American Thoracic Society 5
- Oxygen therapy improves survival in hypoxemic patients and is one of the few interventions shown to modify survival rates, according to the American College of Chest Physicians 4
Pulmonary Rehabilitation
- Exercise training should include a combination of constant load or interval training, strength training, and upper extremity exercise training, as suggested by the American Thoracic Society 1, 2
- Pulmonary rehabilitation should be offered to all patients with significant symptom burden, according to the American College of Chest Physicians 4
Non-Invasive Ventilation
- Non-invasive ventilation (NIV) may be considered for patients with recent hospitalization for acute respiratory failure, as recommended by the American Thoracic Society 5
- For patients with both restrictive lung disease and obstructive sleep apnea, continuous positive airway pressure (CPAP) is indicated, according to the American Academy of Sleep Medicine 5
Surgical and Interventional Options
- For selected patients with advanced emphysema refractory to medical care, surgical or bronchoscopic lung volume reduction may be beneficial, as suggested by the American Thoracic Society 5
- Lung transplantation should be considered for appropriate candidates with progressive disease, according to the International Society for Heart and Lung Transplantation 5
Preventive Measures
- Annual influenza vaccination is recommended for all patients, as suggested by the Centers for Disease Control and Prevention 3
- Pneumococcal vaccinations (PCV13 and PPSV23) are recommended for all patients older than 65 years of age, according to the Centers for Disease Control and Prevention 5
Self-Management Education
- Educational programs should include strategies to minimize dyspnea, as recommended by the American Thoracic Society 1
- Discussion of advance directives should be included in educational programs when appropriate, according to the American College of Chest Physicians 5