What Is Emphysema?

Edited by Guy Slowik MD FRCS. Last updated on June 28th 2011 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. Damage occurs to the tiny airways in the lungs called bronchioles. Bronchioles are joined to alveoli, tiny grape-like clusters of sacs in the lungs where oxygen from the air is exchanged for carbon dioxide from the body. The elastic properties of the lung reside in the tissue around the alveoli. In emphysema:

Because the lungs lose elasticity they become less able to contract. This prevents the alveoli from deflating completely, and the person has difficulty exhaling. Hence, the next breath is started with more air in the lungs. The trapped "old" air takes up space, so the alveoli are unable to fill with enough fresh air to supply the body with needed oxygen.

A person with emphysema may feel short of breath during exertion and, as the disease progresses, even while at rest.

Emphysema is one of several irreversible lung diseases that diminish the ability to exhale. This group of diseases is called chronic obstructive pulmonary disease (COPD). The two major diseases in this category are emphysema and chronic bronchitis, which often develop together. For more information about bronchitis, go to Bronchitis.
Facts About Emphysema

Nearly two million Americans have emphysema. Emphysema ranks 15th among chronic conditions that force people to limit their activities. Cigarette smoking is the primary cause of emphysema. Most people with emphysema are older men. As with lung cancer and other smokingrelated diseases, however, the incidence of emphysema is increasing among women. Emphysema doesn't develop suddenly. Instead, it comes on gradually, usually after

years of exposure to cigarette smoke or some other inhaled irritant. Typically, symptoms of emphysema appear only after 30 to 50 percent of lung tissue is lost. Emphysema rates are highest for men age 65 and older. More people in the Midwest have emphysema than in any other region in the country. Emphysema is an irreversible disease that can be slowed but not reversed or stopped.

What Causes Emphysema?

Edited by Guy Slowik MD FRCS. Last updated on June 28th 2011 Generally, lungs become damaged because of reactions to irritants entering the airways and alveoli. Researchers continue to investigate the factors that may make some people more susceptible to emphysema than others. But there are some clear causes for emphysema:

Cigarette smoking Alpha-1 antitrypsin deficiency

Cigarette Smoking
Cigarette smoking is the major cause of emphysema. When exposed to cigarette smoke, the air sacs of the lungs produce defensive cells, called macrophages, which "eat" the inhaled particles. But macrophages are stimulated to release materials which can destroy the proteins that let the lungs expand and contract, called elastin and collagen. Cigarette smoke also damages the cilia, tiny hair-like projections in the bronchi that "sweep" foreign bodies and bacteria out of the lungs.

Alpha-1 Antitrypsin Deficiency

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. AAT is thought to protect against some of the damage caused by macrophages. In AAT deficiency-related emphysema, the walls of the bronchial tubes and the alveoli are both damaged, often leading to severe disease.
Need To Know:

About 2 out of every 1,000 people have an alpha-1 antitrypsin deficiency. People who smoke and have AAT deficiency are almost certain to develop emphysema.

What Are The Symptoms Of Emphysema?

Edited by Guy Slowik MD FRCS. Last updated on June 28th 2011 The first sign of emphysema is shortness of breath when you are exerting yourself. Eventually, this shortness of breath may occur even when you are at rest. As the disease progresses, the following symptoms which are related to one of the other major lung diseases also caused by smoking - bronchitis - may occur:

Difficulty breathing (dyspnea - this can also be caused by emphysema) Coughing (with or without sputum) Wheezing (this can also be caused by emphysema itself) Excess mucus production A bluish tint to the skin (cyanosis)

Nice To Know: If wheezing occurs, it helps to know whether it is found while breathing in or out (or both). Wheezing at the end of a complete exhalation is usually due to bronchitis. Wheezing that begins early in expiration is usually due to emphysema or the combination of bronchitis and emphysema - COPD. If you only wheeze when you breathe in, you probably have asthma (or, very rarely, a narrowing of your windpipe in your neck). Asthma and emphysema sometimes are confused with each other. One way to tell the difference is to try asthma medications prescribed by your doctor and see if they make a difference. While people with asthma often respond dramatically to medications, people with emphysema usually do not respond to asthma medications and those with COPD may respond somewhat. In some people bronchitis may lead to asthma which is difficult, even for physicians, to distinguish from asthma due to allergy. Nice To Know: Emphysema may affect the brain, too. Low oxygen levels in the blood may mean that the brain is not getting enough oxygen. The end result can be grumpiness, irritability, impaired mental ability. High carbon dioxide levels in the blood can lead to headaches and

sleeplessness.

Emphysema

Definition
Emphysema is a chronic respiratory disease where there is over-inflation of the air sacs (alveoli) in the lungs, causing a decrease in lung function, and often, breathlessness.

Description
Emphysema is the most common cause of death from respiratory disease in the United States, and is the fourth most common cause of death overall. There are 1.8 million Americans with the disease, which ranks fifteenth among chronic conditions that cause limitations of activity. The disease is usually caused by smoking, but a small number of cases are caused by an inherited defect. Normally functioning lungs are elastic, efficiently expanding and recoiling as air passes freely through the bronchus to the alveoli, where oxygen is moved into the blood and carbon dioxide is filtered out. When a person inhales cigarette smoke or certain other irritants, his or her immune system responds by releasing substances that are meant to defend the lungs against the smoke. These substances can also attack the cells of the lungs, but the body normally inhibits such action with the release of other substances. In smokers and those with the inherited defect, however, no such prevention occurs and the lung tissue is damaged in such a way that it loses its elasticity. The small passageways (bronchioles) leading to the alveoli collapse, trapping air within the alveoli. The alveoli, unable to recoil efficiently and move the air out, over expand and rupture. As the disease progresses, coughing and shortness of breath occur. In the later stages, the lungs cannot supply enough oxygen to the blood. Emphysema often occurs with other respiratory diseases, particularly chronic bronchitis. These two diseases are often referred to as one disorder called chronic obstructive pulmonary disease (COPD). Emphysema is most common among people aged 50 and older. Those with inherited emphysema may experience the onset as early as their thirties or forties. Men are more likely than women to develop emphysema, but female cases are increasing as the number of female smokers rises.

Causes and symptoms

Heavy cigarette smoking causes about 80-90% of all emphysema cases. However a few cases are the result of an inherited deficiency of a substance called alpha-1-antitrypsin (AAT). The number of Americans with this deficiency is relatively small, probably no greater than 70,000. Pipe, cigar, and marijuana smoking can also damage the lungs. While a person may be less likely to inhale cigar and pipe smoke, these types of smoke can also impair lung function. Marijuana smoke may be even more damaging because it is inhaled deeply and held in by the smoker. The symptoms of emphysema develop gradually over many years. It is a common occurrence for many emphysema patients to have lost over half of their functioning lung

tissue before they become aware that something is wrong. Shortness of breath, a chronic mild cough (which may be productive of large amounts of dark, thick sputum, and often dismissed as "smoker's cough"), and sometimes weight loss are associated with emphysema. Initially, a patient may only notice shortness of breath when he or she is exercising. However, as the disease progresses, it will occur with less exertion or no exertion at all. Emphysema patients may also develop an enlarged, or "barrel,", chest. Other symptoms may be skipped breaths, difficulty sleeping, morning headaches, increased difficulty breathing while lying down, chronic fatigue, and swelling of the feet, ankles, or legs. Those with emphysema are at risk for a variety of other complications resulting from weakened lung function, including pneumonia.

Diagnosis
A variety of pulmonary function tests may be ordered. In the 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 breathed out after taking a deep breath (vital capacity). With severe emphysema, vital capacity is substantially below normal. Spirometry, a procedure that measures air flow and lung volume, helps in the diagnosis of emphysema. A chest x ray is often ordered to aid in the diagnosis of emphysema, though patients in the early stages of the disease may have normal findings. Abnormal findings on the chest x ray include over-inflation of the lungs and an abnormally increased chest diameter. The diaphragm may appear depressed or flattened. In addition, patients with advanced emphysema may show a smaller or vertical heart. The physician may observe blisters in the lungs and bulging of the accessory muscles of the respiratory system. Late in the disease, an EKG will show signs of right ventricular failure in the heart and increased hemoglobin due to lower levels of oxygen in the patient's blood.

Treatment
Treatment methods for emphysema do not cure or reverse the damage to the lungs. However, they may slow the progression of the disease, relieve symptoms, and help control possibly fatal complications. The first step in treatment for smokers is to quit, so as to prevent any further deterioration of breathing ability. Smoking cessation programs may be effective. Consistent encouragement along with the help of health care professionals as well as family and friends can help increase the success rate of someone attempting to quit. If the patient and the health care team develop and maintain a complete program of respiratory care, disability can be decreased, acute episodes of illness may be prevented, and the number of hospitalizations reduced. However, only quitting smoking has been shown to slow down the progression of the disease, and among all other treatments, only oxygen therapy has shown an increase in the survival rate. Home oxygen therapy may improve the survival times in those patients with advanced emphysema who also have low blood oxygen levels. It may improve the patient's tolerance of exercise, as well as improve their performance in certain aspects of brain function and muscle coordination. The functioning of the heart may also improve with an increased concentration of oxygen in the blood. Oxygen may also decrease insomnia and

headaches. Some patients may only receive oxygen at night, but studies have illustrated that it is most effective when administered at least 18, but preferably 24 hours per day. Portable oxygen tanks prescribed to patients carry a limited supply and must be refilled on a regular basis by a home health provider. Medicare and most insurance companies cover a large proportion of the cost of home oxygen therapy. Patients should be instructed regarding special safety issues involving the transport and presence of oxygen in the home. A variety of medications may be used in the treatment of emphysema. Usually the patient responds best to a combination of medicines, rather than one single drug. Bronchodilators are sometimes used to help alleviate the patient's symptoms by relaxing and opening the airways. They can be inhaled, taken by mouth, or injected. Another category of medication often used is corticosteroids or steroids. These help to decrease the inflammation of the airway walls. They are occasionally used if bronchodilators are ineffective in preventing airway obstruction. Some patients' lung function improves with corticosteroids, and inhaled steroids may be beneficial to patients with few side effects. A variety of antibiotics are frequently given at the first sign of a respiratory infection, such as increased amounts of sputum, or if there has been a change in the color of the sputum. Expectorants can help to loosen respiratory secretions, enabling the patient to more easily expel them from the airways. Many of the medications prescribed involve the use of a metered dose inhaler (MDI) that may require special instruction to be used correctly. MDIs are a convenient and safe method of delivering medication to the lungs. However, if they are used incorrectly the medication will not get to the right place. Proper technique is essential for the medication to be effective. For some patients, surgical treatment may be the best option. Lung volume reduction surgery is a surgical procedure in which the most diseased parts of the lung are removed to enable the remaining lung and breathing muscles to work more efficiently. Preliminary studies suggest improved survival rates and better functioning with the surgery. Another surgical procedure used for emphysema patients is lung transplantation. Transplantation may involve one or both lungs. However, it is a risky and expensive procedure, and donor organs may not be available. For those patients with advanced emphysema, keeping the air passages reasonably clear of secretions can prove difficult. Some common methods for mobilizing and removing secretions include:

Postural drainage. This helps to remove secretions from the airways. The patient lies in a position that allows gravity to aid in draining different parts of the lung. This is often done after the patient inhales an aerosol medication. The basic position involves the patient lying on the bed with his chest and head over the side and the forearms resting on the floor. Chest percussion. This technique involves lightly clapping the back and chest, and may help to loosen thick secretions. Coughing and deep breathing. These techniques may aid the patient in bringing up secretions. Aerosol treatments. These treatments may involve solutions of saline, often mixed with a bronchodilator, which are then inhaled as an aerosol. The aerosols thin and

loosen secretions. A treatment normally takes 10 to 15 minutes, and is given three or four times a day. Patients with COPD can learn to perform a variety of self-help measures that may help improve their symptoms and their ability to participate in everyday activities. These measures include:

Avoiding any exposure to dusts and fumes. Avoiding air pollution, including the cigarette smoke of others. Avoiding other people who have infections like the cold or flu. Get a pneumonia vaccination and a yearly flu shot. Drinking plenty of fluids. This helps to loosen respiratory secretions so they can be brought up more easily through coughing. Avoiding extreme temperatures of heat or cold. Also avoiding high altitudes. (Special precautions can be taken that may enable the emphysema patient to fly on a plane.) Maintaining adequate nutritional intake. Normally a high protein diet taken in many small feedings is recommended.

Alternative treatment
Many patients are interested in whether any alternative treatments for emphysema are available. Some practitioners recommend supplements of antioxidant nutrients. There have also been some studies indicating a correlation between a low Vitamin A levels and COPD, with suggestions that supplements of vitamin A might be beneficial. Aromatherapists have used essential oils like eucalyptus, lavender, pine, and rosemary to help relieve nasal congestion and make breathing easier. The herb elecampane may act as an expectorant to help patients clear mucus from the lungs. The patient should discuss these remedies with their health care practitioner prior to trying them, as some may interact with the more traditional treatments that are already being used.

Prognosis
Emphysema is a serious and chronic disease that cannot be reversed. If detected early, the effects and progression can be slowed, particularly if the patient stops smoking immediately. Complications of emphysema include higher risks for pneumonia and acute bronchitis. Overall, the prognosis for patients with emphysema is poor, with a survival rate for all those with COPD of four years, and even less for emphysema. However, individual cases vary and many patients can live much longer with supplemental oxygen and other treatment measures.

Prevention
The best way to prevent emphysema is to avoid smoking. Even patients with inherited emphysema should avoid smoking, as it especially worsens the onset and severity. If patients quit smoking as soon as evidence of small airway obstruction begins, they can significantly improve their prognosis.

Key terms
Alveoli Small cells or cavities. In the lungs, these are air sacs where oxygen enters the blood and carbon dioxide is filtered out. Pulmonary Related to or associated with the lungs.

Resources

Books
Beers, Mark H., and Robert Berkow, editors. The Merck Manual of Diagnosis and Therapy. Whitehouse Station, NJ: Merck and Company, Inc., 2004.

Periodicals
"Data Mounting on Merits of Lung Volume ReductionSurgery." Family Practice News February 15, 2001: 5. Lewis, Laurie. "Optimal Treatment for COPD." PatientCare May 30, 2000: 60.

Organizations
American Lung Association. 1740 Broadway New York, NY 10019. (212) 315-8700. http://www.lungusa.org. National Emphysema Foundation. 15 Stevens St. Norwalk, CT 06856. http://www.emphysemafoundation.org. National Heart, Lung and Blood Institute. http://www.nhlbi.nih.gov. Gale Encyclopedia of Medicine. Copyright 2008 The Gale Group, Inc. All rights reserved. emphysema /emphysema/ (emf-semah) 1. a pathologic accumulation of air in tissues or organs. 2. pulmonary e.emphysematous atrophic emphysema senile e. bullous emphysema single or multiple large cystic alveolar dilatations of lung tissue. centriacinar emphysema , centrilobular emphysema focal dilatations of respiratory bronchioles rather than alveoli, throughout the lung among normal lung tissue. congenital lobar emphysema overinflation of a lung, usually in early life in one of the upper lobes, with respiratory distress. hypoplastic emphysema pulmonary emphysema due to a developmental anomaly, with fewer and abnormally large alveoli. infantile lobar emphysema congenital lobar e. interlobular emphysema air in the septa between lung lobules. interstitial emphysema air in the peribronchial and interstitial tissues of the lungs. intestinal emphysema pneumatosis cystoides intestinalis. mediastinal emphysema pneumomediastinum. obstructive emphysema that associated with partial bronchial obstruction that interferes with exhalation. panacinar emphysema , panlobular emphysema a type characterized by enlargement of air spaces throughout the acini.

pulmonary emphysema abnormal increase in size of lung air spaces distal to the terminal bronchioles. pulmonary interstitial emphysema (PIE) a condition seen mostly in premature infants, in which air leaks from lung alveoli into interstitial spaces, often because of underlying lung disease or use of mechanical ventilation. senile emphysema overdistention and stretching of lung tissues due to atrophic changes. subcutaneous emphysema air or gas in subcutaneous tissues, usually caused by intrathoracic injury. surgical emphysema subcutaneous emphysema following surgery. vesicular emphysema panacinar e. Dorland's Medical Dictionary for Health Consumers. 2007 by Saunders, an imprint of Elsevier, Inc. All rights reserved. emphysema ( m f -s m , -z -) n. 1. A pathological condition of the lungs marked by an abnormal increase in the size of the air spaces, resulting in labored breathing and an increased susceptibility to infection. It can be caused by irreversible expansion of the alveoli or by the destruction of alveolar walls. Also called pulmonary emphysema. 2. An abnormal distention of body tissues caused by retention of air. em physem atous (-s m -t s, -s m -, -z m -, -z m -) adj. em physe mic adj. & n. The American Heritage Medical Dictionary Copyright 2007, 2004 by Houghton Mifflin Company. Published by Houghton Mifflin Company. All rights reserved. emphysema [emfsm] Etymology: Gk, en + physema, a blowing an abnormal condition of the pulmonary system, characterized by overinflation and destructive changes in alveolar walls. It results in a loss of lung elasticity and decreased gas exchange. When emphysema occurs early in life, it is usually related to a rare genetic deficiency of serum alpha-1-antitrypsin, which inactivates the enzymes leukocyte collagenase and elastase. More common causes are air pollution and cigarette smoking. Acute emphysema may be caused by the rupture of alveoli during severe respiratory efforts, as may occur in acute bronchopneumonia, suffocation, whooping cough, and, occasionally, labor. Patients with chronic emphysema may also have a component of chronic bronchitis. Emphysema also occurs after asthma or tuberculosis, conditions in which the lungs are overstretched until the elastic fibers of the alveolar walls are destroyed. In old age the alveolar membranes atrophy and may collapse, producing large, air-filled spaces and a decreased total surface area of the pulmonary membranes. There are three primary types: centriacinar emphysema, distal acinar emphysema, and panacinar emphysema. -emphysematous, adj. observations The patient may have dyspnea on exertion or at rest, cough, orthopnea, unequal chest expansion, tachypnea, tachycardia, diminished breath sounds caused by air trapping, or, atypically, an elevated temperature and breath sounds if there is an infection. Anxiety, increased PaCO2, restlessness, confusion, weakness, anorexia, hypoxemia, and

respiratory failure are common in advanced cases. Chronic emphysema is characterized by increased anterior-posterior chest diameter secondary to hyperinflation and air trapping and use of accessory muscles. interventions The primary treatment consists of breathing exercises, oxygen administration, and avoiding infection. The airway is kept open, and oxygen is administered to maintain an arterial oxygen saturation of 92%. Bronchodilators, antibiotics, expectorants when bronchitis is also present, methylxanthines, and corticosteroids may be prescribed. Sedation is to be avoided because sedatives depress respiratory function. nursing considerations The patient is taught breathing exercises and encouraged to drink between 2 and 3 L of fluids daily, if not contraindicated by cardiac function. Activity is encouraged to the limit of the patient's tolerance. Fatigue, constipation, and upper respiratory tract infection and irritation are to be avoided. Mechanical ventilation and oxygen therapy may be prescribed for use at home. The patient is taught the adverse role that smoking plays in the disease and is encouraged to stop smoking. Mosby's Medical Dictionary, 8th edition. 2009, Elsevier. emphysema (emfizm ), n 1. a swelling caused by air in the tissue spaces. In the oral and facial regions it may be caused either by air introduced into a tooth socket or gingival crevice with the air syringe, or by blowing of the nose. n 2. a permanent dilation of the respiratory alveoli. Mosby's Dental Dictionary, 2nd edition. 2008 Elsevier, Inc. All rights reserved. emphysema a pathological accumulation of air in tissues. The air may derive from a skin laceration and be drawn in by the movements of muscles. A discontinuity of the tracheal mucosa is a common cause, either by way of laceration or ulceration. Extension from a pulmonary lesion is also common. The syndrome resulting depends on the location of the air. See also pulmonary emphysema and subcutaneous emphysema (below). acute bovine pulmonary emphysema see atypical interstitial pneumonia. alveolar emphysema see pulmonary emphysema (below). bullous emphysema emphysema in which bullae form in areas of lung tissue so that these areas do not contribute to respiration. conjunctival emphysema may occur after head trauma which permits escape of air from the paranasal sinuses. fetal emphysema see emphysematous/putrescent fetus. generalized emphysema widespread distribution of air, including subcutaneous tissues, seen with pneumomediastinum. hypoplastic emphysema pulmonary emphysema due to a developmental abnormality, resulting in a reduced number of alveoli, which are abnormally large. interlobular emphysema

accumulation of air in the septa between lobules of the lungs. interstitial emphysema presence of air in the peribronchial and interstitial tissues of the lungs. intestinal emphysema a condition marked by accumulation of gas under the tunica serosa of the intestine. lobar emphysema emphysema involving less than all the lobes of the affected lung. mediastinal emphysema see pneumomediastinum. orbital emphysema may occur after trauma to the head which permits escape of air from the paranasal sinuses; appears as swelling with crepitus under the conjunctiva or periocular skin. panacinar emphysema, panlobular emphysema generalized obstructive emphysema affecting all lung segments, with atrophy and dilatation of the alveoli and destruction of the vascular bed. pulmonary emphysema distention of the lung caused by overdistention of alveoli and rupture of alveolar walls (alveolar emphysema) and in some cases escape of air into the interstitial spaces (interstitial emphysema). It is a common pathological finding in many diseases of the lung in all species, but also occurs independently, especially in horses, as a principal lesion in chronic obstructive pulmonary disease. It is also a prominent lesion in bovine atypical interstitial pneumonia. It is always secondary to a primary lesion which effectively traps an excessive amount of air in the alveoli. It is characterized clinically by cough, dyspnea, forced expiratory effort and poor work tolerance. A double expiratory effort is a characteristic signhence broken wind. subconjunctival emphysema occurs with fractures involving the paranasal sinuses. subcutaneous emphysema air or gas in the subcutaneous tissues. The characteristic lesion is a soft, mobile swelling which crackles like stiff paper when palpated. There is no pain, nor heat and no ill effects unless the pharyngeal area is sufficiently involved to cause asphyxia. surgical emphysema subcutaneous emphysema following operation. unilateral emphysema emphysema affecting only one lung, frequently due to congenital defects in circulation. vesicular emphysema see panacinar emphysema (above). Saunders Comprehensive Veterinary Dictionary, 3 ed. 2007 Elsevier, Inc. All rights reserved emphysema Internal medicine Accumulation of air in tissueeg, lungs, dermis; pulmonary emphysema is characterized by size of air spaces distal to terminal bronchioles, vital capacity and airway resistance, often with alveolar wall destruction and fibrosis; emphysema commonly is often associated with chronic bronchitis and COPD Mechanisms Atrophy, bronchitis, bronchiolitis, scarring. See Centriacinar emphysema,

Coal workers' pneumoconiosis, COPD, Distal acinar emphysema, Giant bullous emphysema, Panacinar emphysema, Senile emphysema. Cf Focal emphysema. McGraw-Hill Concise Dictionary of Modern Medicine. 2002 by The McGraw-Hill Companies, Inc. Patient discussion about emphysema. Q. Does smoking cause emphysema? I know it sounds like a silly question, as you can read everywhere that smoking damages your lung and cause emphysema and lung cancer, but I know a family with 4 brothers,2 of them not smoking and by the age of 50 both of them had emphysema, and another brothers that doesn't stop smoking but is completely healthy. How can than happen? A. When doctors say that smoking cause emphysema, they doesn't say that you must smoke to get emphysema and that everyone that smokes will eventually develop the disease. You can smoke and never have emphysema, and you can never smoke and have emphysema. The numbers (i.e. the risk) is what makes all the difference... Q. can you ever get better from emphysema? A. Emphysema is a chronic state where the lungs pathologically expand and cause them to lose their compliance during breathing. This is not a reversible state, and usually the lung pathological changes will continue to deteriorate if the lungs are exposed to the same pathogens that caused the initial damage (for example- smoking). However, smoking cessation is known to have benefitial results in slowing down the progress of lung disability and somewhat reversing part of the damage by regression of the inflammatory processes that are related to the emphysema. Q. EMPHYSEMA what type of disease is it? A. It's a chronic lung disease, characterized by destruction of the walls of the air sacs (alveoli) where the blood exchange oxygen and CO2 with the air. The disease leads to obstruction of the airflow, air trapping inside the lungs, and to many long term complications, and eventually to death. It's caused mainly by smoking. Emphysema is a long-term, progressive disease of the lungs that primarily causes shortness of breath. In people with emphysema, the tissues necessary to support the physical shape and function of the lungs are destroyed. It is included in a group of diseases called chronic obstructive pulmonary disease or COPD (pulmonary refers to the lungs). Emphysema is called an obstructive lung disease because the destruction of lung tissue around smaller sacs, called alveoli, makes these air sacs unable to hold their functional shape upon exhalation. It is often caused by long-term exposure to air pollution or smoking.

The term emphysema means swelling and comes from the Greek emphysan meaning inflate, itself composed of en meaning in and physan meaning breath, blast.[1] Classification and external resources

A lateral chest x-ray of a person with emphysema. Note the barrel chest and flat diaphragm. ICD-10 J43. ICD-9 492 DiseasesDB 4190 MedlinePlus 000136 eMedicine med/654 MeSH D011656

Contents
[hide]

1 Classification o 1.1 Congenital lobar emphysema o 1.2 Paraseptal emphysema 2 Signs and symptoms 3 Causes 4 Pathophysiology 5 Diagnosis 6 Management 7 Notable cases 8 Footnotes 9 External links

Classification
Emphysema can be classified into primary and secondary. However, it is more commonly classified by location into panacinary and centroacinary (or panacinar and centriacinar,[2] or centrilobular and panlobular).[3]

Panacinar (or panlobular) emphysema: The entire respiratory acinus, from respiratory bronchiole to alveoli, is expanded. Occurs more commonly in the lower lobes, especially basal segments, and anterior margins of the lungs.[2] Centriacinar (or centrilobular) emphysema: The respiratory bronchiole (proximal and central part of the acinus) is expanded. The distal acinus or alveoli are unchanged. Occurs more commonly in the upper lobes.[2]

Other types include distal acinar and irregular.[2] A special type is congenital lobar emphysema (CLE).

[edit] Congenital lobar emphysema

CLE results in overexpansion of a pulmonary lobe and resultant compression of the remaining lobes of the ipsilateral lung, and possibly also the contralateral lung. There is bronchial narrowing because of weakened or absent bronchial cartilage.[4] There may be congenital extrinsic compression, commonly by an abnormally large pulmonary artery. This causes malformation of bronchial cartilage, making them soft and collapsible.[4] CLE is potentially reversible, yet possibly life-threatening, causing respiratory distress in the neonate.[4]

[edit] Paraseptal emphysema

Paraseptal emphysema is a type of emphysema which involves the alveolar ducts and sacs at the lung periphery. The emphysematous areas are subpleural in location and often surrounded by interlobular septa (hence the name). It may be an incidental finding in young adults, and may be associated with spontaneous pneumothorax. It may also be seen in older patients with centrilobular emphysema. Both centrilobular and paraseptal emphysema may progress to bullous emphysema. A bulla is defined as being at least 1 cm in diameter, and with a wall less than 1mm thick. Bullae are thought to arise by air trapping in emphysematous spaces, causing local expansion.[5]

[edit] Signs and symptoms

Emphysema is a disease of the lung tissue caused by destruction of structures feeding the alveoli, in some cases owing to the action of alpha 1-antitrypsin deficiency. Smoking is one major cause of this destruction, which causes the small airways in the lungs to collapse during forced exhalation. As a result, airflow is impeded and air becomes trapped, just as in other obstructive lung diseases. Symptoms include shortness of breath on exertion, and an expanded chest. People with this disease do not get enough oxygen and cannot remove carbon dioxide from their blood; they therefore exhibit dyspnea (shortness of breath). At first this occurs only during physical activity. Eventually it will occur after any physical exertion. Later the patient may be dyspneic all the time, even when relaxing. Because breathing is difficult, the patient must use accessory muscles to help them breathe; tachypnea (rapid breathing) may occur when they try to extend their exertion. They may have trouble coughing and lowered amounts of sputum. They may also lose weight. The anteroposterior diameter of their chest may increase; this symptom is sometimes referred as "barrel chest." The patient may lean forward with arms extended or resting on something to help them breathe. When lung auscultation and chest percussion is performed a hyperresonant sound is heard.

The patient may also exhibit symptoms of hypoxia-induced cyanosis, or the appearance of a blue to purplish discoloration of the skin, due to increased levels of deoxyhemoglobin in the blood.

[edit] Causes
The majority of all emphysema cases are caused by smoking tobacco. Emphysema cases that are caused by other etiologies are referred to as secondary emphysema. In some cases it may be due to alpha 1-antitrypsin deficiency. Severe cases of A1AD may also develop cirrhosis of the liver, where the accumulated A1AT leads to a fibrotic reaction. Some types of emphysema are considered a normal part of aging and are found in the elderly whose lungs have deteriorated due to age. At about 20 years of age, people stop developing new alveoli tissue. In the years following the cessation of the development of new alveoli, lung tissue can start to deteriorate. This is a normal, natural part of aging in healthy people. Alveoli will die, the number of lung capillaries will decline and the elastin of the lungs will begin to break down causing a loss of pulmonary elasticity. As people age, they will also lose strength and mass in their chest muscles causing these muscles to become weaker. In addition, bones can start to deteriorate and a persons posture can change. Together, all of these age-related manifestations can cause the development of emphysema. Though not all elderly people will develop emphysema, they are all at risk of having decreased respiratory function. Other causes of emphysema can be anything that causes the body to be unable to inhibit proteolytic enzymes in the lung. This could be exposure to air pollution, second hand smoke or other chemicals and toxins.

[edit] Pathophysiology

Pathology of lung showing centrilobular emphysema characteristic of smoking. Closeup of fixed, cut surface shows multiple cavities lined by heavy black carbon deposits. (CDC/Dr. Edwin P. Ewing, Jr., 1973) In normal breathing, air is drawn in through the bronchi and into the alveoli, which are tiny sacs surrounded by capillaries. Alveoli absorb oxygen and then transfer it into the blood. When toxicants, such as cigarette smoke, are breathed into the lungs, the harmful

particles become trapped in the alveoli, causing a localized inflammatory response. Chemicals released during the inflammatory response (e.g., elastase) can eventually cause the alveolar septum to disintegrate. This condition, known as septal rupture, leads to significant deformations of the lung architecture[6][7] (video) that have important functional consequences. The key mechanical event consequent to septal rupture is that the resulting cavity is larger than the sum of the two alveolar spaces (see side figure);

An elastic net distended on finite surface may offer a bidimensional model useful for understanding the pure mechanical consequences of septal rupture. In red is the extra space of the new cavity after septal rupture due to the lung elastic recoil re-arrangement, necessarily at the expenses of the space of the surrounding healthy meshes (alveoli) (VIDEO)[8] in fact because of the lacking mechanical support of the broken septa the lung elastic recoil further enlarges this new space, necessarily at the expenses of the surrounding healthy parenchyma. In other words, as immediate and spontaneous consequence of septal rupture, the elastic lung recoil resets healthy parenchyma expansion at a lower level, in proportion to the amount of septal disruption. The large cavities left by the septal rupture are known as bullae (sin. = bulla). These deformations result in a large decrease of alveoli surface area used for gas exchange, as well as decreased ventilation of the surrounding healthy parenchyma. This results in a decreased Transfer Factor of the Lung for Carbon Monoxide (TLCO). To accommodate the decreased surface area, thoracic cage expansion (barrel chest) and diaphragm contraction (flattening) take place. Expiration, which physiologically depends completely on lung elastic recoil, increasingly depends on the thoracic cage and abdominal muscle action, particularly in the end expiratory phase. Due to decreased ventilation, the ability to exude carbon dioxide is significantly impaired. In the more serious cases, oxygen uptake is also impaired. As the alveoli continue to break down, hyperventilation is unable to compensate for the progressively shrinking surface area, and the body is not able to maintain high enough oxygen levels in the blood. The body's last resort is vasoconstricting appropriate vessels. This leads to pulmonary hypertension, which places increased strain on the right side of the heart, the side responsible for pumping deoxygenated blood to the lungs. The heart muscle thickens in order to pump more blood. This condition is often accompanied by the appearance of jugular venous distension. Eventually, as the heart continues to fail, it becomes larger and blood backs up in the liver.

Patients with alpha 1-antitrypsin deficiency (A1AD) are more likely to suffer from emphysema. A1AT inhibits inflammatory enzymes (such as elastase) from destroying the alveolar tissue. Most A1AD patients do not develop clinically significant emphysema, but smoking and severely decreased A1AT levels (10-15%) can cause emphysema at a young age. The type of emphysema caused by A1AD is known as panacinar emphysema (involving the entire acinus) as opposed to centrilobular emphysema, which is caused by smoking. Panacinar emphysema typically affects the lower lungs, while centrilobular emphysema affects the upper lungs. A1AD causes about 2% of all emphysema. Smokers with A1AD are at the greatest risk for emphysema. Mild emphysema can often develop into a severe case over a short period of time (12 weeks). While A1AD provides some insight into the pathogenesis of the disease, hereditary A1AT deficiency only accounts for a small proportion of the disease. Studies for the better part of the past century have focused mainly upon the putative role of leukocyte elastase (also neutrophil elastase), a serine protease found in neutrophils, as a primary contributor to the connective tissue damage seen in the disease. This hypothesis, a result of the observation that neutrophil elastase is the primary substrate for A1AT, and A1AT is the primary inhibitor of neutrophil elastase, together have been known as the "protease-antiprotease" theory, implicating neutrophils as an important mediator of the disease. However, more recent studies have brought into light the possibility that one of the many other numerous proteases, especially matrix metalloproteases might be equally or more relevant than neutrophil elastase in the development of non-hereditary emphysema. The better part of the past few decades of research into the pathogenesis of emphysema involved animal experiments where various proteases were instilled into the trachea of various species of animals. These animals developed connective tissue damage, which was taken as support for the protease-antiprotease theory. However, just because these substances can destroy connective tissue in the lung, as anyone would be able to predict, doesn't establish causality. More recent experiments have focused on more technologically advanced approaches, such as ones involving genetic manipulation. One particular development with respect to our understanding of the disease involves the production of protease "knock-out" animals, which are genetically deficient in one or more proteases, and the assessment of whether they would be less susceptible to the development of the disease. Often individuals who are unfortunate enough to contract this disease have a very short life expectancy, often 03 years at most

Diagnosis
The diagnosis is usually confirmed by pulmonary function testing (e.g. spirometry); however, X-ray radiography may aid in the diagnosis. A DLCO test may be used to differentiate Emphysema from other types of Obstructive disorders such as Chronic Bronchitis and Asthma. DLCO is a test that measures the ability of gases to diffuse across

the alveolar-capillary membrane. A DLCO will be decreased in Emphysema whereas it will be normal or increased in Asthma and Chronic Bronchitis.

H&E stained lung tissue of end-stage emphysema. RBCs are red, nuclei are purple, other material is pink, and air spaces are white.

Micrograph demonstrating emphysema (left of image - large empty spaces) and lung tissue with relative preservation of the alveoli (right of image). H&E stain.

Axial CT image of the lung of a person with end-stage bullus emphysema.

A severe case of emphysema.

Lung bulla as seen on CXR in a person with severe COPD

[edit] Management
Emphysema is an irreversible degenerative condition. Patients who think they may have contracted the disease are recommended to seek medical attention as soon as possible. The most important measure to slow its progression is for the patient to stop smoking and avoid all exposure to cigarette smoke and lung irritants. Pulmonary rehabilitation can be very helpful to optimize the patient's quality of life and teach the patient how to actively manage his or her care. Emphysema is also treated by supporting the breathing with anticholinergics, bronchodilators, steroid medication (inhaled or oral), effective body positioning (High Fowlers), and supplemental oxygen as required. Treating the patient's other conditions including gastric reflux and allergies may improve lung function. Supplemental oxygen used as prescribed (usually more than 20 hours per day) is the only non-surgical treatment which has been shown to prolong life in emphysema patients. There are lightweight portable oxygen systems which allow patients increased mobility. Patients can fly, cruise, and work while using supplemental oxygen. Other medications are being researched. Lung volume reduction surgery (LVRS) can improve the quality of life for certain carefully selected patients. It can be done by different methods, some of which are minimally invasive. In July 2006 a new treatment, placing tiny valves in passages leading to diseased lung areas, was announced to have good results, but 7% of patients suffered partial lung collapse. The only known "cure" for emphysema is lung transplant, but few patients are strong enough physically to survive the surgery. The combination of a patient's age, oxygen deprivation and the side-effects of the medications used to treat emphysema cause damage to the kidneys, heart and other organs. Surgical transplantation also requires the patient to take an anti-rejection drug regimen which suppresses the immune system, and can lead to microbial infection of the patient. With the discovery of multipotent lung stem cells in 2011, a new treatment option may soon become available. Scientists injected human lung stem cells into mice with damaged lungs. The stem cells formed human bronchioles, alveoli, and pulmonary vessels integrated structurally and functionally with the damaged mouse organ. The May 2011 report in the New England Journal of Medicine [9] concluded that human lung stem cells "have the undemonstrated potential to promote tissue restoration in patients with lung disease".

[edit] Notable cases

Amy Winehouse[10] Paul Avery[11] Don Imus[12] Leonard Nimoy[13]

R. J. Reynolds, R. J. Reynolds Jr., R. J. Reynolds, III,[14] Spencer Tracy[15] Johnny Carson[16][17] Dick York[18]

Occupational dust exposure and chronic obstructive pulmonary disease. A systematic overview of the evidence.
Oxman AD, Muir DC, Shannon HS, Stock SR, Hnizdo E, Lange HJ.

Source
Department of Family Medicine, Clinical Epidemiology & Biostatistics, and Medicine, Faculty of Health Sciences, McMaster University, Hamilton, Ontario, Canada.

Abstract
The object of this study was to assess the relationship between occupational dust exposure and chronic obstructive pulmonary disease (COPD). Studies were identified using MEDLINE (January 1966 to July 1991), SCISEARCH, manual review of reference lists, and personal contact with more than 30 international experts. Studies of COPD, lung function, emphysema, chronic bronchitis, or mortality in workers exposed to nonorganic dust were retrieved. Studies were included if dust exposure was measured quantitatively, and a quantitative relationship between dust exposure and one of the outcomes of interest was calculated while controlling at least for smoking and age. Methodological rigor was assessed, and data regarding the study populations, prognostic factors, and outcomes were extracted independently by two reviewers. Thirteen reports derived from four cohorts of workers met our inclusion criteria. Three of the cohorts were of coal miners and one was of gold miners. All of the studies found a statistically significant association between loss of lung function and cumulative respirable dust exposure. It was estimated that 80 (95% CI, 34 to 137) of 1,000 nonsmoking coal miners with a cumulative respirable dust exposure of 122.5 gh/m3 (considered equivalent to 35 years of work with a mean respirable dust level of 2 mg/m3) could be expected to develop a clinically important (> 20%) loss of FEV1 attributable to dust. Among 1,000 smoking miners the comparable estimate was 66 (95% CI, 49 to 84). The risk of a clinically important loss of lung function attributable to dust among nonsmoking gold miners was estimated to be three times as large as for coal miners at less than one fifth of the cumulative respirable dust exposure (21.3 gh/m3), the maximal exposure observed among the cohort of gold miners. We conclude that occupational dust is an important cause of COPD, and the risk appears to be greater for gold miners than for coal miners.

One possible explanation of the greater risk among gold miners is the higher silica content in gold mine dust.

Comment in

Definition
By Mayo Clinic staff Emphysema

Emphysema occurs when the air sacs in your lungs are gradually destroyed, making you progressively more short of breath. Emphysema is one of several diseases known collectively as chronic obstructive pulmonary disease (COPD). Smoking is the leading cause of emphysema. As it worsens, emphysema turns the spherical air sacs clustered like bunches of grapes into large, irregular pockets with gaping holes in their inner walls. This reduces the surface area of the lungs and, in turn, the amount of oxygen that reaches your bloodstream. Emphysema also slowly destroys the elastic fibers that hold open the small airways leading to the air sacs. This allows these airways to collapse when you breathe out, so the air in your lungs can't escape. Treatment may slow the progression of emphysema, but it can't reverse the damage.

Risk factors
By Mayo Clinic staff Factors that increase your risk of developing emphysema include:

Smoking. Emphysema is most likely to develop in cigarette smokers, but cigar and pipe smokers also are susceptible. The risk for all types of smokers increases with the number of years and amount of tobacco smoked. Age. Although the lung damage that occurs in emphysema develops gradually, most people with tobacco-related emphysema begin to experience symptoms of the disease between the ages of 40 and 60. Exposure to secondhand smoke. Secondhand smoke, also known as passive or environmental tobacco smoke, is smoke that you inadvertently inhale from someone else's cigarette, pipe or cigar. Being around secondhand smoke increases your risk of emphysema.

Occupational exposure to fumes or dust. If you breathe fumes from certain chemicals or dust from grain, cotton, wood or mining products, you're more likely to develop emphysema. This risk is even greater if you smoke. Exposure to indoor and outdoor pollution. Breathing indoor pollutants, such as fumes from heating fuel, as well as outdoor pollutants car exhaust, for instance increases your risk of emphysema.

Complications
By Mayo Clinic staff People who have emphysema are also more likely to develop:

Collapsed lung (pneumothorax). A collapsed lung can be life-threatening in people who have severe emphysema, because the function of their lungs is already so compromised. Heart problems. Emphysema can increase the pressure in the arteries that connect the heart and lungs. This can cause a condition called cor pulmonale, in which a section of the heart expands and weakens. Large holes in the lungs (giant bullae). Some people with emphysema develop empty spaces in the lungs called bullae. Giant bullae can be as large as half the lung. In addition to reducing the amount of space available for the lung to expand, giant bullae can become infected and are more prone to causing a collapsed lung (pneumothorax).

Complications
COPD affects an estimated 9 - 10% of people worldwide in both developed and underdeveloped countries. It is the 4th most common cause of death in the United States, but experts predict that it will be the third leading cause of death in the world by 2020 as the population ages and people continue to smoke. Although COPD has traditionally been considered a man's disease, an increase in women who smoke has caused COPD to skyrocket in women. Women with COPD tend to fare worse than men -- they are more likely to be hospitalized and to die from COPD. They also report more severe symptoms, greater depression, and a worse quality of life than men.

Women appear to be more susceptible to the effects of smoking and pollution, possibly because of hormones or other genetic differences. The good news is that women who stop smoking get their lung function back more quickly than men. The leading cause of death from COPD is respiratory failure. However, patients with mild-to-moderate COPD tend to succumb to cardiovascular disease or lung cancer. This likely occurs from chronic low-grade inflammation, which is involved in all three COPD diseases. Traditionally, physicians have measured the severity of COPD by the amount of air that a person can forcibly exhale in one second (FEV1). The amount decreases as COPD worsens. However, COPD affects other systems and body parts, which provide clues about the severity of the disease. Many physicians now use the BODE index to categorize COPD and predict outcome. BODE stands for body-mass index, degree of airflow obstruction, dyspnea (breathlessness), and exercise capacity as measured in a 6-minute walk test.

Outlook for Patients with Emphysema. If emphysema is detected before causing symptoms, there may be some chance of reversing it, although permanent changes in the alveoli usually occur, even in young smokers. Patients with the inherited form of early-onset emphysema are at risk for early death, unless the disease is treated and its progression halted or slowed. Emphysema patients who experience severe involuntary weight loss (which indicates muscle wasting) have a poorer outlook, regardless of their lung function. Outlook for Patients with Chronic Bronchitis. Chronic bronchitis does not cause as much lung damage as emphysema, although the airways become blocked by mucus plugs, and narrow due to inflammation. Thus poor air exchange causes reduced levels of oxygen, and high carbon dioxide levels. This state of poor gas exchange can lead to serious, life-threatening conditions that include severe breathing difficulty and heart failure.

Acute Exacerbations
Acute exacerbations are episodes that occur when airways suddenly become obstructed and symptoms worsen. Such events are associated with inflammation in the airways and are generally triggered by an infection in the airway or throughout the body. Other factors that can trigger serious lung events:

Certain medications Exposure to irritants in the air Seasonal changes

Acute exacerbations include the following symptoms:

Increased volume of sputum

Sputum that is thicker and darker Worsened shortness of breath that causes the patient to breathe faster and harder. This is the most common and distressing acute symptom.

Acute exacerbations occur, on average, between two and three times a year in patients with moderate-to-severe COPD. In about 80% of the cases, they are triggered by infections. Smokers have more episodes than nonsmokers. Acute exacerbations get better on their own, but they are still the most common cause of hospitalization in these patients, and often require different medications to improve. Frequent acute exacerbations of COPD cause lung function to deteriorate quickly, and patients never recover to the condition they were in before the last exacerbation. In COPD patients who are hospitalized, mortality rates are 11%. Survivors of a first hospitalization have a 50% chance of rehospitalization within 6 months.

Reduced Quality of Life and Mood

Nearly half of patients with COPD report a limitation in daily activities. They have trouble walking up stairs or carrying even small packages. Breathing becomes hard work. More than half of patients with COPD have insomnia. Such impairment in quality of life can negatively affect mood. Almost half of patients with COPD have anxiety, depression, or another psychiatric disorder, compared with 31% of people in the general population. Women with COPD are more susceptible to psychological problems than men. If patients with COPD become anxious or depressed, they may have a poorer outlook than those without these emotional problems. COPD patients with moderate-to-severe depression face a greater 3-year mortality rate than those who experience less depression. Low oxygen levels also can impair mental function and short-term memory. Psychological interventions may be particularly helpful for people with COPD.

Malnourishment
People with COPD often lack good nutrition. Patients with chronic bronchitis tend to be obese. Patients with emphysema tend to be underweight. Loss of weight and muscle mass is associated with a poor outcome in COPD. Good nutrition improves the ability to exercise, which in turn builds muscle strength and lung function. Obese patients with COPD who lose weight sleep better.

Heart Disease
Over time, COPD causes low levels of oxygen (hypoxia) and high levels of carbon dioxide (hypercapnia) in the body. In order to boost oxygen delivery, the body compensates in a number of ways:

Blood vessels in the lung constrict to force blood and oxygen through the circulatory system. This leads to high blood pressure in the lungs (pulmonary hypertension). More red blood cells are produced to increase the blood's oxygen-carrying capacity. The heart rate increases to pump more blood. The rate of breathing increases.

Eventually these activities can lead to very serious and even life-threatening conditions:

Abnormally high blood pressure in the lungs can cause a complication called cor pulmonale, in which the right ventricle of the heart enlarges, eventually leading to heart failure. Chronic bronchitis is associated with a 50% higher risk of death from coronary artery disease, independent of the risks associated with smoking. Patients with prolonged and severe hypoxia and hypercapnia are at risk for acute respiratory failure, which can cause heart rhythm abnormalities or other lifethreatening conditions.

Other Serious Medical Problems Associated with COPD

The smoking that causes COPD is associated with high risks of pneumonia, lung cancer, stroke, and heart attack. Tobacco smoke contains more than 400 substances, many of which are oxidants, metals (such as lead, cadmium, and aluminum), and carcinogens. Nicotine itself may not damage tissues, but it is the chemical that addicts the smoker to tobacco. Lung Cancer. Patients with a 30-year history of smoking and indications of airflow limitation (in other words, most patients with COPD), are at high risk for lung cancer. Sleep Disturbance. About half of all people with severe COPD experience sleep disorders such as sleep-related hypoxia or insomnia. Nocturnal hypoxia, a lack of oxygen during sleep, occurs when breathing is shallowest during rapid-eye-movement (REM) sleep. It may be due to suppression of the cough reflex and a build-up of mucus. Nocturnal hypoxia is treated with overnight oxygen therapy. As COPD worsens, many patients have trouble falling or staying asleep. COPD patients should not use sleep medications. Nighttime oxygen or a change in COPD medications from beta-agonists to anticholinergics can sometimes help restore restful sleep. Osteoporosis. Osteoporosis is a significant problem in patients with COPD. Many conditions associated with COPD, including smoking, vitamin D deficiency, sedentary lifestyle, and the use of corticosteroid medications put people at risk for bone density loss and osteoporosis. Gastroesophageal Reflux (GERD). More than half of patients with severe COPD have GERD, a condition in which stomach acids back up from the stomach into the esophagus.

However, many COPD patients don't report experiencing GERD symptoms such as heartburn. More Information on This Topic

Background Causes Symptoms Diseases With Similar Symptoms Complications Risk Factors Diagnostic Tests Medications Treatment Oxygen-Replacement Therapy Lifestyle Changes Surgical Procedures References News & Features Reporter's File Expert Q & A Questions for Your Doctor Clinical Trials

Background
Chronic obstructive pulmonary disease (COPD) is a condition in which there is limited airflow in the lungs. The disease develops and worsens over time, and although it is not reversible, its progress can be slowed with therapy. Although patients can breathe in normally, changes in the small airways cause the walls to narrow during expiration, making it hard to breathe out. In many patients with COPD, the small sacs where oxygen and carbon dioxide are exchanged are destroyed, gradually starving the body of oxygen. COPD is associated with a set of breathing-related symptoms:

Being out of breath, at first when doing physical activities, but as lung function deteriorates, also at rest Chronic cough Spitting or coughing mucus (phlegm)

The ability to exhale (breathe out) gets worse over time.

The lungs are located in the chest cavity and are responsible for respiration. The alveoli are small sacs where oxygen is exchanged in the lungs. The two major diseases in this category are emphysema and chronic bronchitis, both covered in this report. The third, less common disease, is obstructive bronchiolitis, an inflammatory condition of the small airways. Asthma shares some of the same symptoms, but is a very different disease. People can have asthma and COPD at the same time. Because smoking is the most common cause of both emphysema and chronic bronchitis, these conditions often develop together and frequently require similar treatments and approaches. When chronic bronchitis occurs together with emphysema, it is often difficult for a physician to distinguish between the two.

Emphysema
Emphysema is a disease marked by destruction of the alveoli, grape-like clusters of air sacs at the end of the smallest airways (the bronchioles) in the lung. It generally takes the following course:

The walls of the alveoli become inflamed and damaged. Over time they lose the ability to stretch and shrink (elasticity), and pockets of dead air (called bullae) form in the injured areas. The pockets interfere with the normal working of the lungs by narrowing the airways, trapping air, and making breathing out more difficult. The ability to breathe in is not affected. Until the late stages of the disease, oxygen and carbon dioxide levels remain normal.

Click the icon to see an image of emphysema.

Chronic Bronchitis
Chronic bronchitis is defined as coughing and overproduction of mucus for at least 3 months, during at least 2 consecutive years. In chronic bronchitis, the disease process is generally marked by the following characteristics:

Inflammation of the bronchial tubes (from smoking, air pollution, etc.) causes the production of mucus, which clogs the airways and makes breathing difficult. The mucus is cleared through coughing. Both constant coughing and inflammation can damage the bronchial tubes. The tubes swell and thicken, leaving less room for air flow.

Click the icon to see an image of bronchitis.

The Lungs
The lungs are two spongy organs surrounded by a thin, moist membrane called the pleura. Each lung is made up of smooth, shiny lobes. The right lung has three lobes, and the left has two. About 90% of the lung is filled with air; only 10% is solid tissue. When a person inhales, air travels through the following pathways into the lungs:

Air is carried from the windpipe (trachea) into the lung through flexible airways called bronchi. Like the branches of a tree, bronchi divide into more than a million smaller airways called bronchioles. The bronchioles lead to grape-like clusters of microscopic sacs called alveoli. In each adult lung there are millions of these tiny alveoli. The thin membrane of the alveoli allows oxygen and carbon dioxide to pass to and from capillaries. During deep inhalation, the elastic alveoli unfold and unwind to allow this exchange of gases to occur.

Capillaries, the smallest of the blood vessels, carry blood throughout the body. Red blood cells carry oxygen throughout the body, and return carbon dioxide to the lungs. White blood cells are the critical infection fighters in the body.

Click the icon to see an image of normal lungs.

Causes
Cigarette smoke accounts for more than 80% of all cases of chronic obstructive lung disease. It contains irritants that inflame the air passages, setting off a cascade of biochemical events that damage cells in the lung, increasing the risk for both COPD and lung cancer. Different effects of smoking can lead to emphysema or chronic bronchitis, but smokers generally have signs of both conditions. The specific diagnosis depends on which disease process predominates. Smoking is the major cause of emphysema. In some rare inherited disorders, emphysema can develop in nonsmokers.

Disease Process
The key process leading to emphysema is not well understood. It appears that inflammatory cells (T lymphocytes, neutrophils, and alveolar macrophages) release chemicals called enzymes that attack the tissue in the deepest part of the lung where oxygen and carbon dioxide are exchanged. As a result, the bronchioles detach from the alveoli, and holes appear. Airways become narrowed, and exhaling becomes difficult.

Smoking
The typical COPD patient is a current or former smoker, over age 50, with a pack-a-day habit of more than 20 years. Lung function continues to get worse as the person ages. Smoking is the major cause of COPD worldwide. In underdeveloped countries, smoke, exposure to industrial pollutants in poorly ventilated work areas, and cooking over wood and coal fires are also major contributors. As smoking has become more widespread among women, the incidence of COPD in women has grown proportionally. What was once considered a man's disease now affects a greater number of women. Moreover, women appear to be more susceptible to the effects of smoking and pollution than men. On the positive side, smoking rates in the U.S. are dropping, and the proportion of adults under 55 with COPD is gradually declining. This indicates the high death rate will

eventually level out. In particular, the rate of COPD in young African-Americans is declining significantly. The rate in younger Caucasians is not decreasing as dramatically. Over 80% of people who die from COPD are current or former smokers. The longer a person smokes, the higher the risk for emphysema. Most patients have a history of smoking the equivalent of one pack a year for 20 years, and many have smoked up to the equivalent of 40 years. Once a smoker quits, the rate of lung function loss becomes the same as in a nonsmoker; however, much of the lung damage that occurred during smoking may be irreversible. Emphysema caused by smoking most often occurs in the upper lobes of the lungs. In chronic bronchitis, smoking triggers inflammation that causes damage in the airways. The processes involved include:

Damage to the cilia, the hair-like waving projections that move mucus, bacteria, and foreign particles out of the lungs. When cilia are injured, such agents become trapped in the lungs and can cause infections that lead to chronic bronchitis. Enlargement of the mucus glands in the large airways of the lungs. Overgrowth of smooth muscle cells in the airway.

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. 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.

[Epidemiology of emphysema: analysis by autopsy in a series of elderly patients].

[Article in Japanese] Jinno S, Kida K, Ootsubo K.

Source
Pulmonary Division, Tokyo Metropolitan Geriatric Hospital.

Abstract
Epidemiologic studies indicate that various factors are involved in causing emphysema, although it is uncertain exactly how these factors contribute. Thus the correlation between pathological changes and clinical manifestations was studied. Results of autopsies done on 1940 men and 1791 women from 1978 to 1992 were analyzed retrospectively. Emphysema was graded, from macroscopic findings as follows: none (E 0), slight (E 1), moderate (E 2), and severe (E 3). The severity of anthracocsis was graded as well. Information regarding clinical diagnosis, smoking habits, and available spirometric data were obtained by reviewing the medical records. Prevalence of each grade of emphysema was: in men, E 0-48.6%, E 1-31.6%, E 2-15.8%, and E 3-4.0%; in women, E 0-81.6%, E 1-13.7%, E 2-3.7%, and E 3-0.8%. Pneumonia, lung cancer, and gastric ulcer were significant complications of emphysema, and may have contributed to the cause of death. The effects of various risk factors on the severity of emphysema were evaluated by multiple linear regression analysis. Male sex, age, smoking habit, and grade of the anthracosis were independent factors affecting the development of emphysema. Among them, anthracosis grade and smoking habit were found to be strongly contributing factors. Emphysema grade and FEV1% were significantly correlated, but several patients with moderate or severe emphysema did not show airflow obstruction. Therefore, receiver operating characteristic (ROC) curves were constructed to evaluate the value of the FEV1% in the diagnosis of emphysema. The diagnostic value of the FEV1% alone was low, so a multiple linear regression equation with three factors (sex,

smoking habit, and FEV1%) was constructed.(ABSTRACT TRUNCATED AT 250 WORDS)

Morphological aspects, classification and epidemiology of emphysema.

Dijkman JH.

Abstract
Emphysema is an anatomically defined disorder, which leads to chronic airflow obstruction. Airflow obstruction, however, may also result from bronchial disease. Pulmonary function tests, as applicable in population studies, do not differentiate between anatomical emphysema and other causes of airflow obstruction. Standard radiology of the thorax provides a morphological approach, but is only indicative of emphysema in advanced cases. Post mortem studies, in which emphysema was quantitated, show that age, smoking habits, sex and environment factors are important for the prevalence of pulmonary emphysema.