Bullous Emphysema

Bullous emphysema (BE) is a severe form of emphysema that damages air sacs in your lungs. The damage affects how your lungs exchange oxygen and carbon dioxide. Normally, air sacs (alveoli) bring in oxygen when you breathe in and remove carbon dioxide when you breathe out.

With bullous emphysema, damage to the alveoli causes their walls to stretch and form large pockets of air. Healthcare providers call these air pockets bullae. Lung bullae can be larger than about 1/2 inch (1 centimeter).

BE can affect one or both of your lungs. Having BE in one lung can affect how well your other lung expands. This can make breathing more difficult. If bullae become very large, they can cause your lung to shrink (vanishing lung syndrome).

BE is common. There’s no cure for it. But treatments can help manage symptoms and stop BE from getting worse.

Other names for bullous emphysema (pronounced “BUHL-uhs em-fuh-ZEE-muh”) include:

• Emphysematous bullae
• Giant emphysematous bullae
• Bullous lung disease

How serious is bullous emphysema?

It’s serious. Without treatment, it can cause permanent damage to your lungs that gets worse over time.

What are the symptoms of bullous emphysema?

Some people don’t have symptoms. But if large bullae (giant bullae) develop, they can cause symptoms. The most common one is shortness of breath (dyspnea). Others may include:

• Chest pain or pressure
• A cough that’s usually worse after waking up
• Feeling very tired
• Loss of appetite
• Feeling like you may throw up
• Trouble breathing
• Wheezing
• Barrel chest
• Clubbed fingers
• Swelling

Your healthcare provider may also check for a loud heartbeat due to pulmonary hypertension.

What causes it?

One of the most common causes of bullous emphysema is smoking cigarettes. Long-term (chronic) inflammation from smoking causes your alveoli walls to break down. Bullae then get bigger, which affects the flow of oxygen and carbon dioxide.

Alpha-1 antitrypsin deficiency is also a common cause. This is a genetic disorder that affects your lungs and liver. It affects a protein that helps keep your lungs healthy.

Other, less common causes include:

• Vaping
• Smoking marijuana or crack cocaine
• Intravenous (through your vein) drug use

What are the risk factors?

Anyone can get bullous emphysema. But your risk may be greater if you have certain genetic conditions, including:

• Alpha-1 antitrypsin deficiency
• Ehlers-Danlos syndrome
• Marfan syndrome

You’re also at a greater risk of getting BE if you have HIV.

How to lower your risk

The best way to reduce your risk of developing bullous emphysema is to avoid smoking. If you don’t smoke, don’t start. If you do smoke, talk to a healthcare provider. They can help you quit.

Complications

Bullous emphysema complications may include:

• Collapsed lung (pneumothorax)
• Coughing up blood (hemoptysis)
• Lung infections
• Vanishing lung syndrome

How is bullous emphysema diagnosed?

Your healthcare provider will review your health history and ask about your biological family health history. They’ll also ask about your symptoms and perform a physical exam.

If they suspect bullous emphysema, they may suggest the following tests to help make a diagnosis:

• Pulmonary function testing to see how well your lungs exchange air and whether bullae obstruct airflow
• Imaging tests, including a chest X-ray and CT scan, to see the location, number and size of bullae in your lungs
• Blood tests to check for low hemoglobin levels, signs of respiratory acidosis and signs of alpha-1 antitrypsin deficiency
• Electrocardiogram (EKG/ECG) to look for signs of lung disease affecting your heart

Once your provider diagnoses you with BE, they may also assign a type to describe how much of your lung is affected.

What are the types?

Healthcare providers may use different types to describe how severe bullous emphysema is:

• Type I: There’s only one bulla, and it hasn’t spread to any other alveoli.
• Type II: You have bullae between the pleura and chest wall.
• Type III: Bullae occur throughout your lung.

How is bullous emphysema treated?

Your treatment may include:

• Bronchodilators to help improve airflow and manage symptoms
• Antibiotics or corticosteroids to treat severe symptoms or a lung infection
• Oxygen therapy to raise low oxygen levels

If you have giant bullae, severe symptoms or complications, you may need surgery. Bullous emphysema surgery may include:

• Bullectomy: Your surgeon removes the bullae from your lung using open surgery (thoracotomy) or a minimally invasive procedure (video-assisted thoracic surgery).
• Bronchoscopic lung volume reduction (BLVR): Your surgeon inserts a valve into your lung to intentionally collapse part of your lung. This is a minimally invasive procedure.
• Lung volume reduction surgery (LVRS): Your surgeon removes damaged lung tissue to help the remaining healthy tissue function better.

If you don’t respond to these treatments, your healthcare provider may recommend a lung transplant.

When should I see my healthcare provider?

See your healthcare provider if you have any symptoms of bullous emphysema, like breathing changes, a cough or chest pain. Get help right away if you have symptoms like:

• Bluish or greyish lips or nails (cyanosis)
• Mental changes, including trouble focusing or staying alert
• Feeling like you can’t breathe
• Rapid heartbeat (tachycardia)

What is the prognosis for bullous emphysema?

It’s a lifelong health condition. You can’t reverse the damage, and your lungs won’t heal. But with treatment, you can help stop bullous emphysema from getting worse. Be sure to follow your healthcare provider’s instructions. They can help you stay healthy and prevent serious complications.