What is emphysema?

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Emphysema is a chronic obstructive pulmonary disease (COPD) that presents as an abnormal
and permanent enlargement of air spaces distal to the terminal bronchioles. It frequently occurs
in association with obstructive pulmonary problems and chronic bronchitis. It is unusual for
someone to have pure emphysema unless it is a result of genetic abnormalities. Most people
have some combination of emphysema and chronic bronchitis with varying
degrees
of airway bronchospasm. This condition is commonly referred to as COPD (and in the United
Kingdom, as chronic obstructive lung disease, COLD).
There are three morphological types of emphysema; 1)centriacinar, 2) panacinar, and
3) paraseptal.
1. Centriacinar begins in the respiratory bronchioles and spreads peripherally mainly in the
upper half of the lungs and is usually associated with long-standingcigarette smoking.
2. Panacinar predominates in the lower half of the lungs and destroys the alveolar tissue and is
associated with homozygous alpha-1 antitrypsin deficiency, a genetic disease.
3. Paraseptal emphysema preferentially localizes around the septae of the lungs or pleura,
often associated with inflammatory processes, like prior lung infections.
What are the risk factors for emphysema?
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The major factors that increase the risk for developing emphysema are:
Smoking: Smoking is one of the major risk factors for developing emphysema; the risk
increases as the number of years the person has been smoking increases, and is related to the
amount of tobacco smoked (for example, three cigarettes a day versus a pack and a half per
day); smoking is a major risk factor also for developing lung cancer.
Exposure to secondhand smoke: the risk factors or emphysema increase for people exposed
to secondhand smoke according to the number of years exposed to secondhand smoke, and
the amount of smoke the person is exposed to.
Exposure to fumes or dust in the environment: People that work in close association with
chemical fumes or dusts generated in mining, chemical plants or other industries are higher
risks for developing emphysema; these risks are further increased if the person smokes
tobacco.
Pollution: Air pollution caused by fumes from vehicles, electrical generating plants that use coal
and other fumes produce increases the risk of emphysema.
In the underdeveloped parts of the world, indoor air pollution primarily from open wood
flames used for cooking is the primary mechanism for acquiring emphysema.
What causes emphysema?
As stated previously in the risk factors section, there can be many causes for the development of
emphysema. However, the majority of cases of emphysema (COPD) in the United States and other
countries are caused by exposure to cigarette smoke. Although genetics may play a role, the inflammation
mediated by the body's cells (neutrophils, macrophages and lymphocytes) is usually triggered by
exposure to inflammatory compounds, many of which are found in tobacco smoke. The response of the
+++body's immune system leads to destruction of elastin and other structural elements in the lungs,
ultimately producing areas in the lungs that cannot function normally.
People with alpha-1 antitrypsin deficiency have an inherited autosomal condition that results in increased
breakdown of elastin in the lungs, resulting in COPD (emphysema). When foreign irritants and
substances enter the alveoli, usually by inhalation, an inflammatory process is initiated. Chemical
messages are sent out recruiting white cells to remove this foreign material. These cells release enzymes
that destroy this substance. Normally, these enzymes, often trypsins (protein desolving enzymes) work to
remove this material. The body has anti-trypsin enzymes that destroy the trypsin when the foreign
substance is no more. In the case of the genetic alpha one antitrypsin deficiency, these enzymes continue
to work unabated destroying normal adjacent lung tissue, resulting in emphysema. This is often referred
to as the "innocent bystander" effect.
What are the signs and symptoms of emphysema?
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In most people, emphysema starts to produce signs and symptoms as the person ages; usually symptoms
start developing around age 50, but many can develop symptoms even before age 50.
The onset often develops gradually, and disease is well established before symptoms occur.
The first symptoms frequently vary depending on the degree of emphysema verses bronchitis that the
individual has. If more bronchitis is present, the person may coughproducing sputum and/or acute chest
discomfort also occurs.
As emphysema progresses the most significant symptom becomes breathlessness, especially with any
exertion.Wheezing also may occur. It is common for a middle aged person with emphysema to complain
that they are unable to keep up with their partner when walking.
People with alpha1 antitrypsin deficiency usually experience the above symptoms, but at a much earlier
age and also may have liver dysfunction.


How is emphysema diagnosed?
After obtaining a history and physical exam, especially noting whether or not the patient is or
has been a tobacco smoker, the doctor may recommend several tests.
Usually the first preliminary test is a chest X-ray that helps distinguish emphysema from
other lung problems.
A computerized tomography(CT) scan may be ordered to provide the doctor with more
information. This test allows the physician to see the actual makeup of the lung tissue and
visualize the degree of emphysema.
Additional tests such as lung function spirometry tests that measure how well a person can
move air in andout of their lungs is often ordered.
More elaborate testing determines how well gases diffuse through the lung tissue. This test
is called thediffusing capacity test, and is a physiologic test of underlying emphysema in
patients with obstructed airflow, as measured by spirometry. In addition, blood taken from an
artery can determine how well the patient's blood is being oxygenated by the lungs, and how
well carbon dioxide is being removed.
A non-invasive test of oxygenation is the oximeter. This is a small device usually place on a
finger that measures a person's oxygen saturation and pulse.
The diagnosis of emphysema is based on the results of these tests. Often, your primary doctor
will refer you to a pulmonary specialist for any additional diagnostic tests and treatments.
What is the treatment for emphysema?
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The first treatment for patients with emphysema is smoking cessation if they are
currently smoking tobacco. This is a difficult lifestyle change for many patients, and without
support from their doctors, family members, and friends; this most important treatment will likely
fail. The best way to accomplish this difficult task is outlined in the "quitting smoking" section. In
addition, there is pharmacological and surgical
therapy
available for emphysema patients and these therapies will be discussed in the next sections.
Quitting smoking
Quitting smoking is the most effective therapy for people with emphysema. Consequently,
successful cessation is a major goal for people with COPD/emphysema. This goal usually can
be reached with cooperation between the doctor, patient, family members, and friends. Quitting
smoking usually requires patient
education
about the risks of smoking, methods to help the patient quit smoking (including a target date to
quit), and follow-up support. Many people will relapse, but they still should be encouraged to try
to change their lifestyle and attempt to quit again.
Many people may benefit from both self-help and groupsmoking cessation programs. Patients
need to understand that nicotine is responsible for their addiction to smoking and may benefit
from a program that allows them to slowly withdraw from nicotine addiction. There are several
types of pharmacological interventions such asnicotine chewing gum, transdermal nicotine
patches, and other treatments such as varenicline (Chantix) and Zybanthat may be used to help
the patient overcome their nicotine addiction.
Emphysema medications
Most patients with the emphysema utilize bronchodilators that dilate airways and decrease
airflow resistance. Some bronchodilators are short-acting while others are long-acting. However,
these drugs provide symptomatic relief, but do not stop the progression of the disease nor do
they decrease mortality. Short acting Beta-2 agonists (SABA) relax bronchial smooth muscle
(such as albuterol[Ventolin, Proventil, Proventil-HFA, AccuNeb, Vospire, ProAir], levalbuterol
[Xopenex], and metaproterenol]).
Other forms are more long acting and usually need to be taken once or twice a day and
include salmeterol(Serevent), indacaterol (Arcapta Neohaler), and formoterol (Foradil); often
referred to as long acting Beta agonists, LABA.
Another long acting group of medications acting through a different mechanism of
bronchodilation are called long acting muscarinic antagonists or LAMA. These include drugs like
tiotropium and aclidinium.
Some patients have benefited from the use of theophylline(Respbid, Slo-Bid, Theo-24,
Theolair); however because of its narrow therapeutic range and potential for toxicity, it is
infrequently utilized.
Corticosteroids (for example, fluticasone [Flonase, Feramyst] or budesonide [Entocort EC,
Uceris ER]) usually inhaled (ICS, inhaled corticosteroid) are used to decrease the inflammatory
components of COPD/emphysema; they are usually added to the treatment protocol that
includes a long acting bronchodilator. Often these drugs are administered in combination,
LABA and ICS and include Advair, Symbicort, Dulera, and Breo.
Roflumilast, a selective phosphodiesterase inhibitor, is used to improve shortness of breath and
increase lung function in some people with emphysema, but most evidence shows that
it can reduce exacerbations.
Finally, antibiotics are often used to treat the infections that frequently occur with people with
COPD/emphysema because of the body's poor ability to keep mucous and other debris from
blocking airways. The most utilized antibiotics are amoxicillin (Amoxil, Trimox, Moxatag,
Larotid), doxycycline, trimethoprim/sulfamethoxazole(Bactrim, Septra),
and azithromycin (Zithromax, Zmax). There is data supporting the use of chronic azithromycin to
reduce COPD exacerbations, and that this may be more related to anti-inflammatory properties
of this antibiotic and not so much its ability to kill bacteria.
Oxygen therapy may be an important part of therapy to help people improve their function and
duration of their lives. Patients with moderate to severe emphysema should be tested to see if
their oxygen levels fall to abnormal levels with sleep and exercise. If so, supplemental oxygen
should be supplied. Many people with emphysema own personal finger oximeters that inform
them when their oxygen levels drop, especially during exercise.

Quitting smoking
Quitting smoking is the most effective therapy for people with emphysema. Consequently,
successful cessation is a major goal for people with COPD/emphysema. This goal usually can
be reached with cooperation between the doctor, patient, family members, and friends. Quitting
smoking usually requires patient
education
about the risks of smoking, methods to help the patient quit smoking (including a target date to
quit), and follow-up support. Many people will relapse, but they still should be encouraged to try
to change their lifestyle and attempt to quit again.
Many people may benefit from both self-help and groupsmoking cessation programs. Patients
need to understand that nicotine is responsible for their addiction to smoking and may benefit
from a program that allows them to slowly withdraw from nicotine addiction. There are several
types of pharmacological interventions such asnicotine chewing gum, transdermal nicotine
patches, and other treatments such as varenicline (Chantix) and Zybanthat may be used to help
the patient overcome their nicotine addiction.
Emphysema medications
Most patients with the emphysema utilize bronchodilators that dilate airways and decrease
airflow resistance. Some bronchodilators are short-acting while others are long-acting. However,
these drugs provide symptomatic relief, but do not stop the progression of the disease nor do
they decrease mortality. Short acting Beta-2 agonists (SABA) relax bronchial smooth muscle
(such as albuterol[Ventolin, Proventil, Proventil-HFA, AccuNeb, Vospire, ProAir], levalbuterol
[Xopenex], and metaproterenol]).
Other forms are more long acting and usually need to be taken once or twice a day and
include salmeterol(Serevent), indacaterol (Arcapta Neohaler), and formoterol (Foradil); often
referred to as long acting Beta agonists, LABA.
Another long acting group of medications acting through a different mechanism of
bronchodilation are called long acting muscarinic antagonists or LAMA. These include drugs like
tiotropium and aclidinium.
Some patients have benefited from the use of theophylline(Respbid, Slo-Bid, Theo-24,
Theolair); however because of its narrow therapeutic range and potential for toxicity, it is
infrequently utilized.
Corticosteroids (for example, fluticasone [Flonase, Feramyst] or budesonide [Entocort EC,
Uceris ER]) usually inhaled (ICS, inhaled corticosteroid) are used to decrease the inflammatory
components of COPD/emphysema; they are usually added to the treatment protocol that
includes a long acting bronchodilator. Often these drugs are administered in combination,
LABA and ICS and include Advair, Symbicort, Dulera, and Breo.
Roflumilast, a selective phosphodiesterase inhibitor, is used to improve shortness of breath and
increase lung function in some people with emphysema, but most evidence shows that
it can reduce exacerbations.
Finally, antibiotics are often used to treat the infections that frequently occur with people with
COPD/emphysema because of the body's poor ability to keep mucous and other debris from
blocking airways. The most utilized antibiotics are amoxicillin (Amoxil, Trimox, Moxatag,
Larotid), doxycycline, trimethoprim/sulfamethoxazole(Bactrim, Septra),
and azithromycin (Zithromax, Zmax). There is data supporting the use of chronic azithromycin to
reduce COPD exacerbations, and that this may be more related to anti-inflammatory properties
of this antibiotic and not so much its ability to kill bacteria.
Oxygen therapy may be an important part of therapy to help people improve their function and
duration of their lives. Patients with moderate to severe emphysema should be tested to see if
their oxygen levels fall to abnormal levels with sleep and exercise. If so, supplemental oxygen
should be supplied. Many people with emphysema own personal finger oximeters that inform
them when their oxygen levels drop, especially during exercise.

Pulmonary rehabilitation for emphysema
Pulmonary rehabilitation involves methods to improve the patient's quality of life by keeping
airways open and preventing or reducing secondary complications such as infections and
recurrent respiratory symptoms. Pulmonary rehabilitation involves input fromdoctors and nurses,
dietitians, respiratory
therapists
, exercise physiologists, and many others. The goal of pulmonary rehabilitation is to educate the
patient and family about the disease process, encourage routine exercise increasing in graded
increments, smoking cessation, medications and medical management, respiratory and chest
physiotherapy, and exercises to improve breathing. In addition, the program should offer
psychological and social support for the patient. Pulmonary rehabilitation can teach patients how
better to control their disease and live a more vibrant and enjoyable life.
Surgery for emphysema
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Bullectomy, the removal of bullae (thin-walled air filled areas that may compress normal lung
tissue) is one method to reduce some of the symptoms of emphysema/COPD.
Lung volume reduction surgery is another surgical technique. It may be an option for patients
with severe emphysema symptoms that do not respond to attempts at medical therapy. In this
technique, about 20% to 30% of tissue from both lungs is removed; the area removed is
usually the lung tissue sections that have minimal or no function.
Finally, lung transplantation is a possibility for certain selected patients. Patients with
COPD/emphysema are the largest category of patients that undergo lung transplantation.
What are the stages of emphysema?
In general, there are four stages of emphysema; they are as follows:
Stage I: mild obstruction; treatment is with short-acting bronchodilators and reduction of risk
factors
Stage II: moderate obstruction; risk reduction, long-acting bronchodilators, short-acting
bronchodilator as needed and cardiopulmonary rehabilitation
Stage III: severe obstruction; risk reduction, short and long-acting bronchodilators,
cardiopulmonary rehabilitation, and inhaled glucocorticoids as needed
Stage IV: very severe obstruction and/or evidence of chronic respiratory failure, all the above
for stage III, and likely long-term oxygen therapy and consideration of surgical options section.

What is the life expectancy and outlook for someone with emphysema?
Unfortunately, there have been no large studies to determine emphysema's effect on life
expectancy. In general, the life expectancy may be related to the stage of emphysema, but the
prognosis often varies widely between two people in the same stage. The outlook for someone
with emphysema is often good to fair because COPD/emphysema is a slowly progressive
disease. However, reducing risk factors and appropriate treatment may increase the outlook for
many individual patients. Patients that undergo lung transplant have, in general, about a five-
year increase in life expectancy after surgery.