A condition of the lung characterized by increase beyond the normal in the size of air spaces distal to the terminal bronchiole (those parts containing alveoli), with destructive changes in their walls and reduction in their number. Clinical manifestation is breathlessness on exertion, due to the combined effect (in varying degrees) of reduction of alveolar surface for gas exchange and collapse of smaller airways with trapping of alveolar gas in expiration; this causes the chest to be held in the position of inspiration (“barrel chest”), with prolonged expiration and increased residual volume. Symptoms of chronic bronchitis often, but not necessarily, coexist. Two structural varieties are panlobular (panacina) emphysema and centrilobular (centriacinar) emphysema; paracicatricial, paraseptal, and bullous emphysema are also common. Syn: pulmonary emphysema.
Terminology related to emphysema:
Congenital lobar emphysema.
Diffuse obstructive emphysema.
Increased markings emphysema.
Unilateral lobar emphysema.
Alveolar duct emphysema.
Emphysema is a lung disease that reduces the ability of the lungs to expel air, a process which depends upon the natural rubber-band-like quality or elastic properties of the lungs. Emphysema is a type of chronic obstructive airway disease, or COPD. People with COPD have limitations in the flow of air through their airways. Emphysema involves the gradual destruction of alveoli in the lungs. The alveoli are air spaces where oxygen is exchanged with carbon dioxide in the blood.
Emphysema is lung disease that occurs when the tiny air sacs in the lungs are damaged, usually as a result of long-term smoking. Once this damage has occurred, it cannot be repaired. It causes difficulty breathing and shortness of breath that gets worse over time. Emphysema is a respiratory disease in which the alveoli, the tiny air sacs in the lungs, become enlarged. This enlargement causes the alveoli walls to break down, or rupture, narrowing the airways. The narrowed airways make it difficult to breathe in oxygen and exhale carbon dioxide. Emphysema gradually destroys the lung tissue. Emphysema and chronic bronchitis, which often occur together, cause a condition known as chronic obstructive pulmonary disease. Emphysema permanently enlarges and irreversibly damages the alveoli, damages the ends and walls of the smallest bronchioles (the tiny breathing tubes that branch off from the trachea and bronchi), and diminishes the elasticity of the lungs. Emphysema ranks 15th among chronic diseases that contribute to limitation of physical activity. About 44% of those with emphysema report that their daily activities have been limited by the condition.
Chronic obstructive pulmonary diseases, including emphysema, are the fourth leading cause of death in the world. The long-term effects depend on how severe the disease is when diagnosed and if a reduced exposure to the lung irritant occurs. The most effective treatment is quitting smoking. This will typically result in a modest improvement or no further decline in function.
People with emphysema have a higher mortality than those with normal lung function. Causes of death include respiratory failure, lung infections such as pneumonia and influenza, and other diseases related to smoking. These include cancer, heart disease, and stroke. Eventually, severe shortness of breath will limit the person's normal daily activities. People who continue to smoke will have worsening shortness of breath. They may have panic attacks when they are unable to get enough air in and out. People with advanced emphysema are often incapacitated. They are short of breath even when confined to a chair or bed.
Emphysema patients are at increased risk of contracting recurrent respiratory infections and lung cancer, and are at high risk for respiratory and coronary failure and for cor pulmonale, enlargement and strain on the right side of the heart. (Emphysema makes the heart work harder to keep the lungs supplied with blood because of damage to the lungs' circulatory system and other tissue damage.) This condition, which can also be caused by living at high altitudes for prolonged periods, often leads to failure of the right ventricle. Emphysema is a very serious disease that is greatly complicated and worsened by any type of lung infection.
The prevention of emphysema is closely linked to the prevention of smoking. The only risk factor for this disease that you can control is the smoking of cigarettes. Those who are daily smokers put themselves and their health at increasing risk with every pack of cigarettes and with every year they continue to smoke.
Flare-ups of emphysema can be prevented by taking medications as prescribed and seeking medical care for any signs or symptoms of respiratory infection or shortness of breath. Also, if you have emphysema, you should keep current on vaccines that can prevent respiratory infection. It is important to obtain the pneumococcal vaccine every 5 years and the influenza virus vaccine every year, before flu season.
Typically, the first symptoms of emphysema occur in heavy smokers in their mid-50s: Include
Late, severe symptoms include rapid, labored breathing and persistent craving for air (air hunger), even during rest or after minimal physical activity.
Patients are often very thin (a wasted look called "cachexia"), have pinkish skin, and tend to breathe through pursed lips.
Involuntary weight loss and muscle wasting are characteristic of COPD and have a poor outlook. Symptoms of alpha-1 antitrypsin-related emphysema tend to appear between the ages of 30 and 40. As with standard emphysema, they include:
The main early symptom is shortness of breath with light physical activity. Coughing is usually minor, and coughs produce little sputum (phlegm).
Inability to exercise without becoming winded.
Shortness of breath after physical activity.
Causes and Risk factors:
Cigarette smoking is the major cause of emphysema, accounting for more than 80 percent of all cases. Emphysema occurs most often in people older than age 40 who have smoked for many years. Long-term exposure to secondhand smoke also may play a role. Smoking stresses the natural antioxidant defense system of the lung, allowing free radicals to damage tissue down to the cellular level. When cigarette smoke is inhaled, 80 to 90 per cent remains in the lungs and causes irritation, increased mucus production and damage to the deep parts of the lungs. Eventually mucus and tar clog up the air tubes, causing chronic bronchitis and emphysema.
People who a deficiency of a protein called alpha-1 antitrypsin (AAT) are at a higher risk of developing severe emphysema. Alpha-1 antitrypsin deficiency (AAT deficiency) is an inherited condition and occurs in varying degrees. The deficiency leads to A1AD-related emphysema when the liver produces insufficient AAT to control a natural enzyme known as neutrophil elastase. Though neutrophil elastase plays an important role in fighting bacteria and cleaning up dead lung tissue, it eventually causes irreversible damage to the alveoli by damaging or destroying their elastic fibers if there is not enough AAT to neutralize it. For AAT-deficient individuals who smoke, the risk of developing emphysema is much greater than for the general population. A1AD-related emphysema usually strikes people in their thirties or forties and is very rarely seen in children.
Among other causes of emphysema are industrial pollutants, aerosol sprays, non-tobacco smoke, internal-combustion engine exhaust, and physiological atrophy associated with old age (senile emphysema). Physical damage caused by an accident and followed by scarring can give rise to scar emphysema; severe respiratory efforts can rupture alveoli in cases of near suffocation, whooping cough, labor (child-bearing), and acute bronchopneumonia. Tuberculosis and asthma can also give rise to lung overstretching, severely damaging the elastic fibers of the alveoli walls and bringing on emphysema. High altitude is associated with higher death rates among those suffering with COPD, but is not a proven causative factor at this time. Areas of high poverty also experience higher mortality rates among those suffering from chronic obstructive airway disease, possibly a reflection of inadequate medical care.
Genetics: Generally, only 15 - 20% of all smokers develop emphysema. Other factors, such as genetic abnormalities, may need to be present to make people more likely to develop airway damage. However, other than alpha-1 antitrypsin deficiency, few genetic factors have been associated with emphysema or COPD in multiple research studies.
Alpha 1-Antitrypsin Deficiency (A1AD): About 1 out of every 5,000 to 7,000 people have an inherited condition called alpha 1-antitrypsin deficiency (A1AD), which can cause emphysema. People with this disorder do not have enough of the protective enzyme AAT. Without enough AAT, early and progressive damage occurs in both the walls of the alveoli and the airways leading to them.
Because smoke is a major toxin and deactivates any amounts of AAT that do remain, smokers with AAT deficiency have almost no chance of escaping emphysema. Nonsmokers are also at high risk, however. Emphysema in people with A1AD develops in people as young as 30 years old, who are usually of Northern European descent.
Screening tests are now available to detect the genetic defect that causes A1AD. Couples in which one or both partners have a family history of the disease may wish to be tested for the deficiency, so they may take protective measures for themselves and any future children they may have. If the condition is present in the family, testing the children is important.
Bacteria and Viruses: Certain bacteria, particularly Streptococcus pneumoniae, Haemophilus influenzae, and Moraxella catarrhalis, are common in the lower airways of nearly half of chronic bronchitis patients.
However, the role of bacteria, viruses, and other organisms in causing chronic symptoms and inflammation is unclear. Some experts believe that a low-level infection in the lungs may trigger an inflammatory reaction that continues to produce acute attacks.
The primary risk factor for the development of emphysema is tobacco abuse. Cigarette smoke contributes to this disease process in 2 ways. It destroys lung tissue, which is the cause of the obstruction, and it causes inflammation and irritation of airways that can cause the disease to get worse. It is not possible to predict in any individual smoker who will develop emphysema, how long it will take, or how many cigarettes one must smoke. In fact, most smokers do not develop symptomatic emphysema, suggesting that most people have sufficient reserve capacity in the lung to get by despite any damage.
Air pollution acts in a similar manner to cigarette smoke. The pollutants cause inflammation in the airways, leading to lung tissue destruction. 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. In a few individuals, genetic factors such as a reduced level or activity of protective enzymes in the lung (such as occurs in alpha1-antiprotease deficiency) may result in emphysema even in the absence of smoking, but this is much rarer.
Diagnosis of emphysema begins with a medical history and physical examination.
Medical and Personal History: The doctor will request a history that evaluates the patient's risk factors. Risk factors include:
Exposure to industrial pollutants.
Low exercise capacity (such as trouble climbing stairs or difficulty walking for more than a certain distance).
Past and present smoking
Physical Examination: Appearance. No changes in physical appearance are usually present with mild-to-moderate COPD. In advanced COPD, patients with emphysema may be wasted and thin, with normal-colored pink skin, while those with chronic bronchitis may have bluish lips and fingers, be obese, and may have swollen feet and legs. Breathing may be rapid and shallow, done through pursed lips, with prolonged exhales.The patient will be asked to cough and produce sputum, if possible.
Chest Examination: The physician will next perform a simple examination of the chest area with a stethoscope to listen for:
Diminished or distant breath sounds.
Signs of pulmonary hypertension.
Wheezing or gurgling sounds.
Crepitations, a noise resembling a paper bag being rumpled.
Other findings may include:
Flat elevated pressure in the veins.
Breathlessness when the patient lies.
Pulmonary Function Tests (Spirometry): The best tests for diagnosing COPD and seeing how well it responds to treatment are pulmonary function tests. The gold-standard test for patients with respiratory symptoms is spirometry. Spirometry measures the volume and force of air as it is exhaled from the lungs. It measures airway obstruction, can identify COPD early, and the results are standardized so they are always consistent.
The patient is asked to breathe in and to exhale forcefully into an instrument. This is repeated several times. The force of the air is then measured. From the results, the physician determines two important values:
The forced vital capacity (FVC): FVC is the maximum volume of air that can be exhaled (breathed out) with force, and is an indicator of lung size, elasticity, and how well the air passages open and close.
The forced expiratory volume in one second (FEV1): FEV1 is the maximum volume of air exhaled in 1 second. Airflow is considered to be limited if the outflow of forced exhalation stays low over the course of 1 second. People with COPD have a decline in FEV1 over time. FEV1 is measured as "percent of predicted:"
Tests for Measuring the Ability of the Lung to Exchange Oxygen and Carbon Dioxide:
Arterial Blood Gas: The physician may request an arterial blood gas test to determine the amount of oxygen and carbon dioxide in the blood (its saturation). Low oxygen (hypoxia) and high carbon dioxide (hypercapnia) levels often indicate chronic bronchitis, but not always emphysema. A blood gas analysis that shows very low oxygen levels is useful for determining which patients would benefit from oxygen therapy (see below). This procedure typically involves drawing blood from an artery in the wrist.
Pulse Oximetry Test: A safe and painless test for measuring oxygen in the blood is called pulse oximetry, which involves placing a probe on the finger or ear lobe. The probe emits two different lights, and the amount of each light the blood absorbs is related to how much oxygen the red blood cells carry. This test measures only oxygen in the blood, however, and not carbon dioxide. Results should be taken together with other tests to determine the need for medication or oxygen therapy.
Carbon Monoxide Diffusing Capacity: The lung carbon monoxide diffusing capacity (DLCO) test determines how effectively gases are exchanged between the blood and airways in the lungs. Patients should not eat or exercise before the test, and they should not have smoked for 24 hours. The patient inhales a mixture of carbon monoxide, helium, and oxygen and holds his or her breath for about 10 seconds. The gas levels are then analyzed from the exhaled breath. Results can help physicians differentiate emphysema from chronic bronchitis and asthma. Patients with emphysema have lower DLCO results, indicated by a reduced ability to take up oxygen. Such results are also important in helping to determine appropriate candidates for lung reduction surgery. Carbon monoxide levels that are 20% or less than predicted values pose a very high risk for poor survival.
Exhaled Breath: The measurement of nitric oxide (NO) in exhaled breath can be a simple method of diagnosing COPD and monitoring the effects of therapy. In most patients with COPD, no levels are below normal. Levels above normal in a patient with COPD indicate that the person also has asthma.
Chest X-Rays: Chest x-rays are often performed, but they are not very useful for detecting early COPD. By the time an x-ray reveals the disease, the patient is well aware of the condition.
Clear signs of COPD include the following:
A flattened diaphragm.
A smaller heart; if heart failure is present, however, the heart becomes enlarged and there may not be signs of overinflated lungs.
Exaggerated lung inflation in upper areas.
Larger amounts of air in the lower lungs in patients with A1AD-related emphysema. Chest x-rays are rarely useful for diagnosing chronic bronchitis, although they sometimes show mild scarring and thickened airway walls.
Abnormally large amounts of air spaces in the lung.
Computed Tomography: Computed tomography (CT) scans can accurately assess the severity of COPD and may be used to determine the size of the air pockets (bullae) in the lungs. In one study, 3-dimensional CT was used to visualize the airways in a manner in which their diameter could be measured. In this study, airflow limitation in COPD was seen to be related to the dimensions of the small, rather than the large, airways.
Lung function tests can identify emphysema in Stage 0, even before the individual has symptoms. In early stages of emphysema, the only result may be dysfunction of the small airways. Patients with emphysema may show an increase in the total amount of air that is in the lungs (total lung capacity), but a decrease in the amount of air that can be moved in and out of the lungs with one deep breath in and exhale out (vital capacity). Lung function tests measure how much air a person can take in with a deep breath. A physical examination may reveal characteristic sounds in the lungs and, in some patients, a peculiar rounding of the finger nails is observed.
A peak flow meter is a small, hand-held device that measures the severity of breathing impairment at a given moment. The patient takes a deep breath and blows into the machine as hard and long as possible. Arterial blood gas tests measure how well the lungs are oxygenating the blood stream and removing carbon dioxide from it, and pulse oximetry uses light waves to measure blood oxygen levels. Serum alpha-1-antitrypsin levels can be confirmed by blood workup, while urine pH test, pulmonary ventilation-perfusion scan, and chest MRI may all provide valuable indicators to a medical doctor or respiratory therapist. The symptoms of emphysema are very similar to congestive heart failure, sarcoidosis, tuberculosis and other lung diseases and care must be taken by the examining doctor to identify the correct disease.
Lung function tests can give the doctor specific information about how the lungs are working mechanically. These tests involve having you breathe into a tube that is connected to a computer or some other monitoring device, which can record the necessary information. The tests measure how much air your lungs can hold, how quickly your lungs can expel air during expiration, and how much reserve capacity your lungs have for increased demand, such as during exercise.
If you have a family history of alpha-1-antitrypsin deficiency, the doctor may wish to send a blood test to evaluate for this genetic disease.
Blood tests may also be used to check your white blood cell count, which can sometimes indicate an acute infection. This information can be used with the chest x-ray to evaluate for pneumonia, bronchitis, or other respiratory infections that can make emphysema worse.
Another blood test that may be helpful, especially in the hospital setting, is called the arterial blood gas. This test helps doctors determine how much oxygen and carbon dioxide are in your blood. This may show the severity of your emphysema.
A chest x-ray helps the doctor to identify changes in your lung that may indicate emphysema. The x-ray also may show the presence of an infection or a mass in the lung (such as a tumor) that could explain your symptoms. Shortness of breath has many causes. The chest x-ray is considered by most doctors to be the quickest and easiest test to begin to separate the different possible causes and formulate a diagnosis.
Treatment for emphysema can take many forms. Different approaches to treatment are available. Generally, a doctor will prescribe these treatments in a step-wise approach, depending on the severity of your condition.
Stop smoking: Although not strictly a treatment, most doctors make this recommendation for people with emphysema (and everyone). Quitting smoking may halt the progression of the disease and should improve the function of the lungs to some extent. A doctor may be able to prescribe medications to help in breaking the addiction and can also recommend behavioral therapies, such as support groups. You and your doctor should work to find an approach that result in the successful end to cigarette smoking and, in the process, the beginning of improved lung function and quality of life.
Bronchodilating medications: These medications, which cause the air passages to open more fully and allow better air exchange, are usually the first medications that a doctor will prescribe for emphysema. In very mild cases, bronchodilators may be used only as needed, for episodes of shortness of breath.
If you have some degree of shortness of breath at rest, a doctor may prescribe the albuterol to be given at regularly scheduled intervals, either through the MDI, or by nebulization. Nebulization involves breathing in liquid medication that has been vaporized by a continuous flow of air (in much the same way a whole-room vaporizer causes liquid droplets to enter the air by the flow of air through water). Nebulized albuterol may be prescribed once scheduled doses via inhaler are no longer adequate.
Ipratropium bromide (Atrovent) is another bronchodilating medication that is used for relatively mild emphysema. Similar to albuterol, it is available in both an inhaler and as a liquid for nebulization. Unlike albuterol, however, ipratropium bromide is usually given in scheduled intervals. Therefore, it is not usually prescribed for "rescue" purposes. Atrovent lasts longer than albuterol, however, and often provides greater relief.
Methylxanthines (Theophylline) and other bronchodilating medications are available that have varying properties that may make them useful in certain cases. Another fairly common medication is available in a tablet form. Theophylline (Theo-Dur, Uniphyl) is a medication given orally. It can have a sustained effect on keeping air passageways open. Theophylline levels must be monitored by blood tests. Too much theophylline can produce an overdose; too little, and there will not be enough relief of shortness of breath. In addition, other drugs can interact with theophylline, altering the blood level without warning. For this reason, doctors now prescribe theophylline very carefully. If you take theophylline, take the medication as prescribed and check with your doctor before starting any new medication.
The most common bronchodilator for mild cases of emphysema is albuterol (Proventil or Ventolin). It acts quickly, and 1 dose usually provides relief for 4-6 hours. Albuterol is most commonly available as a metered-dose inhaler or MDI, and this is the form that will be used most often for mild emphysema, with intermittent shortness of breath. When used for this purpose, some people refer to their albuterol inhaler as a "rescue" medication. It acts to rescue them from a more serious attack of shortness of breath.
Steroid medications: They decrease inflammation in the body. They are used for this effect in the lung and elsewhere and have been shown to be of some benefit in emphysema. However, not all people will respond to steroid therapy. Steroids may either be given orally or inhaled through an MDI or another form of inhaler.
Antibiotics: These medications are often prescribed for people with emphysema who have increased shortness of breath. Even when the chest x-ray does not show pneumonia or evidence of infection, people treated with antibiotics tend to have shorter episodes of shortness of breath. It is suspected that infection may play a role in an acute bout of emphysema, even before the infection worsens into a pneumonia or acute bronchitis.
Oxygen: If you have shortness of breath and go to a hospital's emergency department, you often are given oxygen. It may even be necessary to give oxygen by placing a tube in your windpipe and allowing a machine to assist your breathing. In some cases, it may be necessary for you to receive oxygen at home as well. There are home-based oxygen tanks available and portable units that enable you to be mobile and engage in normal day-to-day activities.
No treatment can reverse or stop emphysema, but steps can be taken to relieve symptoms, treat complications and minimize disability. The first step in treatment for smokers is to quit, so as to prevent any further deterioration of breathing ability. Physicians may recommend smoking-cessation programs for them. Many patients are also taught controlled coughing techniques to help remove excess mucus from the lungs, which could promote infection. This involves two short, sharp coughs to help expel mucus. Mild exercise may be ordered to help improve breathing. Patients are often instructed on exercise techniques designed to strengthen muscles involved in breathing, as well as other breathing techniques.
Commonly used medications include bronchodilators, which relax the bronchial muscles so the airways are widened and it's easier to breathe. Bronchodilators include albuterol (Proventil, Ventolin and other brand names), salmeterol (Serevent) or ipratropium (Atrovent). These medications are taken via hand-held inhalers or machine-driven nebulizers, which create a fine mist that can be inhaled. Theophylline (sold under several brand names) is a pill form of a bronchodilator. Because it can interact with medications and cause side effects, it is used less often than the inhaler medications. Bronchodilators help to open the bronchial tubes in your lungs and reduce shortness of breath, wheezing and cough.
Steroids called corticosteroids also can help to reduce inflammation in the lungs. During an acute flare-up of symptoms, they often are given in pill form or by injection. Inhaled corticosteroids or pills may be given continually to help control the inflammation of chronic bronchitis. However, these types of drugs usually aren't prescribed for long-term use because of their side effects.
Antibiotics are often prescribed for people with emphysema who have increased shortness of breath. Even when the chest x-ray does not show pneumonia or evidence of infection, people treated with antibiotics tend to have shorter episodes of shortness of breath. It is suspected that infection may play a role in an acute bout of emphysema, even before the infection worsens into a pneumonia or acute bronchitis.
Oxygen therapy has been proven to increase life expectancy in people with emphysema who have below-normal blood oxygen levels. Oxygen usually is administered through a plastic tube (nasal cannula) worn under the nostrils. The oxygen either is stored in metal cylinders, or is purified from air by an electrical machine (an oxygen concentrator). A number of lightweight, portable devices are now available that allow those who need oxygen to leave their homes for hours at a time. Some people with emphysema need oxygen only at night. Because supplying oxygen at home is very expensive, most medical insurance companies have strict requirements to qualify for home oxygen.
Proper nutrition includes the increase of unprocessed foods such as fruits and vegetables and high-quality protein and the decrease of fried foods, alcohol, refined carbohydrates, and processed foods. Also, it is wise to eliminate the use of all mucous producing foods like dairy and gluten-containing grains. If sound nutrition is important in preventing emphysema, it is crucial once the disease has been diagnosed. Malnutrition may increase the risk of respiratory failure in patients with COPD.
Volume reduction surgery has been used with increasing frequency to help improve pulmonary function in patients with advanced emphysema. In the procedure, parts of the lungs are removed to reduce overall volume and improve efficiency of the remaining lung structure. Volume reduction surgery can improve the lung function and quality of life in patients with severe emphysema who are carefully selected based on known factors. Lung transplantation has become a better option for patients with severe emphysema. Transplantation may involve one or both lungs. Statistics show that survival after lung transplantation has been reported at 77% at 2 years and 75% at 3 years. Lung transplantation is a risky and expensive procedure and donor organs may not be available. Once the disease progresses to acute respiratory failure, a patient may be hospitalized and advanced inhalation therapy may be required.
Medicine and medications:
Here is a list of drugs and medications that are commonly prescribed to treat COPD & Emphysema:
Corticosteroids or steroids.
Note: The following drugs and medications are in some way related to, or used in the treatment. This service should be used as a supplement to, and NOT a substitute for, the expertise, skill, knowledge and judgment of healthcare practitioners.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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