Emphysema Types, Stages, Causes, Symptoms, Diagnosis, Prevention, Treatment, FAQs

Emphysema is a long-term, progressive disease of the lungs that primarily causes shortness of breath due to over-inflation of the alveoli (air sacs in the lung).

In people with emphysema, the lung tissue involved in the exchange of gases (oxygen and carbon dioxide) is impaired or destroyed.  Emphysema is included in a group of diseases called a chronic obstructive pulmonary disease or COPD (pulmonary refers to the lungs).

Emphysema is called an obstructive lung disease because airflow on exhalation is slowed or stopped. After all, over-inflated alveoli do not exchange gases when a person breaths due to little or no movement of gases out of the alveoli.

Emphysema changes the anatomy of the lung in several important ways. This is due to in part to the destruction of lung tissue around smaller airways. This tissue normally holds these small airways, called bronchioles, open, allowing air to leave the lungs on exhalation. When this tissue is damaged, these airways collapse, making it difficult for the lungs to empty and the air (gases) becomes trapped in the alveoli.

Normal lung tissue looks like a new sponge. Emphysematous lung looks like an old used sponge, with large holes and a dramatic loss of “springy-ness” or elasticity. When the lung is stretched during inflation (inhalation), the nature of the stretched tissue wants to relax to its resting state.

In emphysema, this elastic function is impaired, resulting in air trapping in the lungs. Emphysema destroys this spongy tissue of the lung and also severely affects the small blood vessels (capillaries of the lung) and airways that run throughout the lung. Thus, not only is airflow affected but so is blood flow. This has a dramatic impact on the ability of the lung not only to empty its air sacs called alveoli (pleural for alveolus) but also for blood to flow through the lungs to receive oxygen.

COPD as a group of diseases is one of the leading causes of death in the United States. Unlike heart disease and other more common causes of death, the death rate for COPD appears to be rising.

Cigarette smoking is by far the most dangerous behavior that causes people to develop emphysema, and it is also the most preventable cause. Other risk factors include a deficiency of an enzyme called alpha-1-antitrypsin, air pollution, airway reactivity, heredity, male sex, and age.

The importance of cigarette smoking as a risk factor for developing emphysema cannot be overemphasized. Cigarette smoke contributes to this disease process in two ways. It destroys lung tissue, which results in the obstruction of airflow, and it causes inflammation and irritation of airways that can add to airflow obstruction.

• Destruction of lung tissue occurs in several ways. First, cigarette smoke directly affects the cells in the airway responsible for clearing mucus and other secretions. Occasional smoking temporarily disrupts the sweeping action of tiny hairs called cilia that line the airways. Continued smoking leads to longer dysfunction of the cilia. Long-term exposure to cigarette smoke causes the cilia to disappear from the cells lining the air passages. Without the constant sweeping motion of the cilia, mucous secretions cannot be cleared from the lower respiratory tract. Furthermore, smoke causes mucous secretion to be increased at the same time that the ability to clear the secretions is decreased. The resulting mucus buildup can provide bacteria and other organisms with a rich source of food and lead to infection.
• The immune cells in the lung, whose job it is to prevent and fight infection, are also affected by cigarette smoke. They cannot fight bacteria as effectively or clear the lungs of the many particles (such as tar) that cigarette smoke contains. In these ways, cigarette smoke sets the stage for frequent lung infections. Although these infections may not even be serious enough to require medical care, the inflammation caused by the immune system constantly attacking bacteria or tar leads to the release of destructive enzymes from the immune cells.
• Over time, enzymes released during this persistent inflammation lead to the loss of proteins responsible for keeping the lungs elastic. In addition, the tissue separating the air cells (alveoli) from one another also is destroyed. Over years of chronic exposure to cigarette smoke, the decreased elasticity and destruction of alveoli leads to the slow destruction of lung function.
• Alpha-1-antitrypsin (also known as alpha-1-antiprotease) is a substance that fights a destructive enzyme in the lungs called trypsin (or protease). Trypsin is a digestive enzyme, most often found in the digestive tract, where it is used to help the body digest food. It is also released by immune cells in their attempt to destroy bacteria and other material. People with an alpha-1-antitrypsin deficiency cannot fight the destructive effects of trypsin once it is released in the lung. The destruction of tissue by trypsin produces similar effects to those seen with cigarette smoking. The lung tissue is slowly destroyed, thus decreasing the ability of the lungs to perform appropriately. The imbalance that develops between trypsin and antitrypsin results in an “innocent bystander” effect. Foreign objects (e.g. bacteria) are trying to be destroyed but this enzyme destroys normal tissue since the second enzyme (antiprotease) responsible for controlling the first enzyme (protease) is not available or is poorly functioning. This is referred to as the “Dutch” hypothesis of emphysema formation.
• Air pollution acts in a similar manner to cigarette smoke. The pollutants cause inflammation in the airways, leading to lung tissue destruction.
• Close relatives of people with emphysema are more likely to develop the disease themselves. This is probably because the tissue sensitivity or response to smoke and other irritants may be inherited. The role of genetics in the development of emphysema, however, remains unclear.
• Abnormal airway reactivity, such as bronchial asthma, has been shown to be a risk factor for the development of emphysema.
• Men are more likely to develop emphysema than women. The exact reason for this is unknown, but differences between male and female hormones are suspected.
• Older age is a risk factor for emphysema. Lung function normally declines with age. Therefore, it stands to reason that the older the person, the more likely they will have enough lung tissue destruction to produce emphysema.

It is important to emphasize that COPD is often not purely emphysema or bronchitis, but varying combinations of both.

• Frequent lung infections
• A lot of mucus
• Wheezing
• Reduced appetite and weight loss
• Fatigue
• Blue-tinged lips or fingernail beds, or cyanosis, due to a lack of oxygen
• Anxiety and depression
• Sleep problems
• Morning headaches due to a lack of oxygen, when breathing at night is difficult

Other conditions share many of the symptoms of emphysema and COPD, so it is important to seek medical advice.

Emphysema Treatment

Treatment of COPD and emphysema aims to stabilize the condition and prevent complications through the use of medication and supportive therapy.

Supportive therapy includes oxygen therapy and helps with smoking cessation.

Drug therapies

The main type of medication used for COPD and emphysema are inhaled bronchodilators to relieve symptoms.

These help by relaxing and opening the air passages in the lungs.

Bronchodilators that are supplied through the inhalers include:

• Beta-agonists, which relax bronchial smooth muscle and increase mucociliary clearance
• Anticholinergics, or antimuscarinics, which relax bronchial smooth muscle.

These drugs are equally effective when regularly used to improve lung function and increase exercise capacity.

There are short-acting and long-acting drugs, and these can be combined.

The choice depends on individual factors, preferences, and symptoms.

Examples include albuterol, formoterol, indacaterol, and salmeterol.

Corticosteroid drugs, such as fluticasone, may also help. The steroids are inhaled as an aerosol spray. They can help relieve symptoms of emphysema associated with asthma and bronchitis.

Corticosteroids may help people with poorly controlled symptoms who regularly experience exacerbations despite using a bronchodilator.

In patients who continue to smoke, corticosteroids do not alter the course of the disease, but they can relieve symptoms and improve short-term lung function in some patients.

Used alongside bronchodilators, they can reduce the frequency of attacks.

However, there is a long-term risk of side-effects that include osteoporosis and cataract formation.

Oxygen therapy

As emphysema progresses and respiratory function declines, independent breathing becomes more difficult.

Oxygen therapy improves oxygen delivery to the lungs. Oxygen can be supplemented by using a range of devices, some of them for home use.

Options include electrically driven oxygen concentrators, liquid oxygen systems, or cylinders of compressed gas, depending on needs and how much time the person spends outdoors or at home.

Oxygen therapy can be administered 24 hours a day or 12 hours at night.

It prolongs life for people with advanced COPD and emphysema.

Patients will be monitored for oxygen saturation to prevent oxygen toxicity.

Air travel may create the need for supplemental oxygen due to the lower flight cabin air pressure.

Surgery

People with severe emphysema sometimes undergo surgery to reduce lung volume or carry out lung transplantation.

Lung volume reduction surgery removes small wedges of the damaged, emphysematous, lung tissue.

This is thought to enhance lung recoil and to improve the function of the diaphragm. In severe cases, this can improve lung function, exercise tolerance, and quality of life.

Lung transplantation improves the quality of life, but not life expectancy, for people with severe emphysema.

Lifelong drug therapy is necessary to prevent the immune system from rejecting the new tissue. One or both lungs may be transplanted.

Treatment of exacerbations

Complications can be managed using drug and oxygen therapy. Antibiotics can help in cases of bacterial infection.

Most exacerbations are treated with corticosteroid drugs, such as prednisone, and oxygen therapy.

Opioid drugs may relieve severe coughing and pain may be relieved by opioid drugs.

In 2014, scientists at the University of Texas Medical Branch in Galveston succeeded in growing human lungs using stem cells. In the future, this could offer hope for people with emphysema and other lung conditions.

Pulmonary rehabilitation and lifestyle management

Pulmonary rehabilitation is a program of care for people with emphysema.

It aims to help people improve their lifestyle by quitting smoking, following a healthful diet, and getting some exercise.

Drinking plenty of water can help keep the airways clear by loosening the mucus.

In winter, avoiding cold air can prevent muscular spasms. A scarf around the mouth or a cold-air face mask may help.

These changes may not alter the overall course of the illness, but they can help people live with the condition, and improve exercise capacity and quality of life.

Exercises that can help improve breathing include diaphragmatic breathing, purse-lip breathing, and deep breathing.

Frequently Asked Questions about Emphysema

What is the life expectancy of a person with emphysema?

Current smokers with stage 1 COPD have a life expectancy of 14.0 years or 0.3 years lower. Smokers with stage 2 COPD have a life expectancy of 12.1 years or 2.2 years lower. Those with stage 3 or 4 COPD have a life expectancy of 8.5 years or 5.8 years lower.

Can your lungs heal from emphysema?

There’s no cure for emphysema, but treatments are available to relieve symptoms and prevent further lung damage.

Can you live for 20 years with COPD?

The American Lung Association reports that COPD is the third leading cause of death in the United States, but as a chronic, progressive disease, most patients will live with the disease for many years. The disease is not curable, yet it is possible to achieve some level of normalcy despite its challenges.

What happens if you have emphysema?

Emphysema is a lung condition that causes shortness of breath. In people with emphysema, the air sacs in the lungs (alveoli) are damaged. Over time, the inner walls of the air sacs weaken and rupture — creating larger air spaces instead of many small ones.