From the Guidelines
Bullous lung disease is primarily caused by smoking, which damages the lungs and leads to the formation of air-filled sacs called bullae. These bullae develop when the walls of the air sacs (alveoli) in the lungs weaken and rupture, creating larger air spaces that reduce the lung's ability to function properly. While smoking is the most common cause, other factors can contribute to bullous lung disease, including alpha-1 antitrypsin deficiency (a genetic disorder), intravenous drug use, and certain infections 1. The disease process involves an imbalance between proteases (enzymes that break down proteins) and antiproteases in the lungs, leading to destruction of lung tissue. This imbalance is worsened by oxidative stress from cigarette smoke and inflammatory processes.
Key Factors
- Smoking is the primary cause of bullous lung disease
- Alpha-1 antitrypsin deficiency is a genetic disorder that can contribute to the disease
- Intravenous drug use and certain infections can also contribute to bullous lung disease
- The formation of bullae reduces the surface area available for gas exchange and can lead to symptoms such as shortness of breath, decreased exercise tolerance, and in severe cases, respiratory failure.
Treatment Options
- Smoking cessation is a crucial step in managing bullous lung disease
- Bronchodilators can help improve lung function and relieve symptoms
- In some cases, surgical intervention to remove large bullae that compress functioning lung tissue may be necessary, as suggested by the National Emphysema Treatment Trial 1. The value of lung volume reduction surgery for other COPD phenotypes is controversial, but patients with severe COPD and giant bullae may experience significant improvements in symptoms, health status, lung mechanics, exacerbation rates, and even survival with lung volume reduction surgery 1.
From the Research
Causes of Bullous Lung Disease
- Bullous lung disease can be caused by a combination of genetic and environmental factors, including alpha-1 antitrypsin deficiency (AATD) and smoking 2, 3, 4.
- AATD is an inherited disorder characterized by low serum levels of alpha-1 antitrypsin, which disrupts the protease-antiprotease balance in the lungs and allows proteases to destroy lung matrix and alveolar structures 3.
- Smoking can exacerbate the progression of bullous lung disease in individuals with AATD, as it increases the decline in lung function and the risk of developing emphysema and chronic obstructive pulmonary disease (COPD) 2, 3, 4.
- The Z variant of AATD is rare in certain populations, such as American blacks, and its presence can contribute to the development of bullous lung disease 2.
- Other factors, such as environmental pollutants and poor nutrition, may also play a role in the development and progression of bullous lung disease, although the evidence is less clear 5.