Bullous Emphysema

What is bullous emphysema?

Bullous emphysema (BE) is a type of emphysema, a lung disease. Emphysema involves damage to the walls of the air sacs (alveoli) in your lungs. Alveoli bring in oxygen when you breathe in and transport carbon dioxide when you breathe out.

Bullous emphysema occurs when there’s damage to your alveolar walls, which stretch to form large pockets of air (bullae). These air pockets can measure larger than 1 centimeter (cm).

Bullae affect how your lungs work. They make it more difficult for your alveoli to exchange oxygen and carbon dioxide. You can have bullous emphysema in one or both lungs. But having BE in one lung can impact the expansion of your other lung, making it more difficult to breathe.

If the bullae become very large, they can cause lung shrinkage and present as vanishing lung syndrome. Although BE can cause permanent damage to your lungs, treatment can help you manage the condition and increase your lifespan.

Who might have bullous emphysema?

People of every age, race and sex can have bullous emphysema. It’s commonly associated with chronic obstructive pulmonary disease (COPD), a smoking-related lung disease. However, you’re at higher risk of developing BE if you have certain genetic conditions, including:

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

Having human immunodeficiency virus (HIV) can also increase your risk of bullous emphysema.

How common is bullous emphysema?

Worldwide, more than 5% of people and more than 12% of adults over age 30 have bullous emphysema. BE is the third leading cause of death in the U.S.

What causes bullous emphysema?

Bullous emphysema can result from chronic obstructive pulmonary disease (COPD). Cigarette smoking is the top cause of BE. Chronic inflammation from smoking results in a breakdown of your alveoli walls. This causes bullae to enlarge, which impacts the flow of oxygen and carbon dioxide.

Bullous emphysema can also be caused by:

• Crack cocaine or marijuana smoking.
• Intravenous (through your vein) drug use.

The genetic conditions listed above cause the walls of your alveoli to become weak and result in damage and bullous emphysema.

What are the symptoms of bullous emphysema?

The main symptom of BE is shortness of breath. Other common symptoms include:

• Chest pain or pressure.
• A cough that’s often worse in the morning.
• Fatigue.
• Loss of appetite.
• Nausea.
• Trouble breathing.

Some people with bullous emphysema don’t have symptoms. If large bullae (called giant bullae) develop, they can cause more symptoms.

Your healthcare provider may look for the following signs when they examine you:

• A barrel-shaped chest.
• Clubbed fingers.
• A loud heartbeat sound due to pulmonary hypertension.
• Swelling (edema).
• Wheezing.

How is bullous emphysema diagnosed?

Your healthcare provider will first ask about your symptoms, health history and family medical history. They’ll also do a physical exam.

If your provider suspects bullous emphysema, they’ll do pulmonary function testing. These tests measure how well your lungs exchange air and whether bullae have obstructed your airflow.

As part of this testing, your provider may do spirometry. During this test, you breathe through a tube into a machine (spirometer). Spirometry looks at the amount of air that passes into and out of your lungs as you breathe.

Your provider may also suggest imaging tests to look for enlarged or damaged alveoli. These tests may include:

• Chest X-ray, to see whether you have bullae and where they are in your lungs.
• CT scan, to see the location, number and size of bullae.

Your provider may also suggest tests, including:

• Blood tests, to check for acidic blood due to a buildup of carbon dioxide in your lungs (respiratory acidosis), or low oxygen levels.
• Complete blood count (CBC), to see if you have low hemoglobin levels.
• Electrocardiogram (EKG), to look for signs of lung disease affecting your heart, including right ventricular hypertrophy and right atrial enlargement.

Your provider may also test you for alpha-1 antitrypsin deficiency using blood tests.

Is there a cure for bullous emphysema?

Unfortunately, there’s no cure for bullous emphysema. But there are treatments to help you better cope with the symptoms and help stop BE from getting worse.

How is bullous emphysema treated?

Your provider may recommend medications called bronchodilators to help manage your symptoms. You usually breathe these medications in through an inhaler. Bronchodilators help improve airflow to and from your lungs and relieve shortness of breath by relaxing the muscles in your airways.

If you have severe symptoms or a lung infection, you may also need to take antibiotics or corticosteroids for a short time.

If your oxygen levels are low, your provider may suggest supplemental oxygen therapy. This supplies your body with extra oxygen through a tube inside your nostrils or a face mask.

You may need surgery if you have giant bullae, severe shortness of breath or complications of BE, such as:

• Collapsed lung (pneumothorax).
• Coughing up blood (hemoptysis).
• Lung infection.

Types of surgery for bullous emphysema include:

• Bullectomy.Your surgeon removes the bullae from your lungs 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 lungs using a minimally invasive procedure to intentionally collapse a part of your lung.
• Lung volume reduction surgery (LVRS). A surgeon removes damaged lung tissue in order to help the remaining tissue function better.

If your condition doesn’t respond to any of the treatments above, your provider may refer you for a lung transplant if appropriate.

How can I reduce my risk of bullous emphysema?

You can reduce your risk of BE by not smoking or by quitting smoking. Your provider can work with you to find techniques to help you quit.

What can I expect if I have bullous emphysema?

Bullous emphysema is a lifelong health condition. Once your lungs become damaged, they won’t heal. But the sooner you start treating BE, the sooner you can help stop it from becoming worse.

How do I take care of myself with bullous emphysema?

You can continue to take care of yourself by managing your symptoms. Finding treatments that work for you will greatly improve your quality of life.

To stay healthy and prevent serious health complications, make sure you get a:

• Flu vaccine once a year to reduce your risk of flu (influenza).
• Pneumococcal vaccine to lower your risk of pneumonia.

When should I see my healthcare provider?

You should see your healthcare provider if you notice any signs of bullous emphysema or have any new symptoms. Seek immediate medical attention if you have symptoms such as:

• Bluish or greyish color in your lips or nails (cyanosis).
• Difficulty staying mentally alert.
• Increased shortness of breath.
• Rapid heartbeat (tachycardia).

A note from Cleveland Clinic

Not being able to catch your breath can be very scary. And if you have bullous emphysema (BE), you may often feel like you’re struggling to breathe. Smoking is the main cause of BE, and having certain genetic conditions can also increase your risk. Quitting smoking is the best thing you can do to stop the progression of bullous emphysema. Your provider can also suggest treatments that can help you breathe easier and make your day-to-day life more manageable.