emphysema

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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 are1.8 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 fourties Men are more likely than women to develop emphysema, but female cases are increasing as the number of female smokers rises.

— Deanna Swartout-Corbeil, RN

Dictionary: em·phy·se·ma   (ĕm'fĭ-sē'mə, -zē'-)

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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.
2. An abnormal distention of body tissues caused by retention of air.

[Greek emphūsēma, inflation, from emphūsān, to blow in : en-, in; see en–² + phūsān, to blow (from phūsa, bellows, bladder).]

A disorder of pulmonary inflation characterized by enlargement and destruction of the air spaces. The key element in this definition is the word destruction for it implies the irreversible loss of a given area of the pulmonary parenchyma. Certain variants of this condition do not necessarily imply irreparable destruction of pulmonary tissue but rather overdistention of air spaces, and consequently are not properly classified as emphysema.

Generalized emphysema probably has many causes; most share chronic bronchiolitis as a factor. Narrowing at this level would cause retention of air, leading to dilatation and rupture of alveolar septa. Increasing attention is being given to heavy cigarette smoking and air pollution as contributing factors. Given the dilatation of the air spaces, the total air space in the lungs is increased. However, the lungs cannot be properly emptied and are functionally impaired.

Emphysema, if widespread, will cause very serious limitation in physical activity. Many cases, however, are compatible with long survival. Complications of severe emphysema include right heart failure (cor pulmonale), respiratory acidosis, and rupture of bullae with development of pneumothorax.

The important variants of emphysema are as follows. Centrilobular emphysema affects predominantly respiratory bronchioles without involvement of the more peripheral elements. In diffuse vesicular emphysema, the most common form, all elements of the respiratory unit (respiratory bronchiole, alveolar ducts, alveolar sacs, and alveoli) are dilated. Senile emphysema was formerly applied to barrel-chested elderly people; however, functional impairment is, in most cases, inconspicuous. This condition is also known as aging lung.

A degenerative disease associated with chronic coughing. It is fairly common in the elderly. The tissue in their airways loses its elasticity, trapping air in the lungs. This effectively reduces breathing capacity and causes breathlessness. Emphysema is exacerbated by smoking but is sometimes alleviated by taking sensibly graded exercise. However, exercise will not cure the condition.

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.

Definition

Emphysema is a progressive, incurable chronic lung condition. The air sacs (alveoli) are destroyed and oxygen uptake is restricted due to the loss of elasticity of lung tissue.

Description

As of 1998 there were an estimated two million people suffering from emphysema in America. Between three and five percent were attributed to genetic factors, the remainder being a result of environmental pollution, with smoking ranking far and away as the main cause.

Normally functioning lungs are elastic, and efficiently expand and recoil as air passes freely through their passageways (bronchus) to the alveoli, where oxygen is moved into the blood and carbon dioxide is filtered out. When a person inhales cigarette smoke or airborne pollutants, 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.

When individuals are exposed to pollution over a long period of time the lung tissue is damaged in such a way that it loses its elasticity. When damage has occurred to the alveoli, sufferers have difficulty making a complete exhalation, which causes residual volume—air trapped inside the lungs. With the passage of time, this causes the chest to permanently expand and become barrel shaped. As the disease progresses, increasingly more effort is needed to breathe. Emphysema frequently occurs with one or more other respiratory diseases, such as bronchitis and asthma. It is one of the diseases that are collectively referred to as chronic obstructive pulmonary disease (COPD). As a cause of death, it ranks fourth after heart disease, cancer and stroke.

Causes & Symptoms

People who smoke or live in polluted atmospheres are possible candidates for emphysema. People with a genetic defect (alpha 1-antitrypsin deficiency) are also at risk. The early stages of emphysema may go undiagnosed, but the main symptoms are breathlessness, blueness of the lips and fingernails, and exhaustion. Sufferers of chronic bronchitis and asthma are also at risk. People who develop emphysema as a result of their work often develop asthma prior to symptoms of their condition.

Emphysema is traditionally a disease suffered by miners, particularly coal miners, as the fine dust that results from mining attacks the alveoli over a period of time. Most miners suffer from emphysema to some degree after a lifetime "down in the pit." In fact, emphysema is sometimes referred to as miner's lung or black lung.

The situation has somewhat improved in recent years due to awareness of the causes of emphysema and improved work conditions for many workers. Others who may be at risk for emphysema include sand blasters, metal grinders, anyone whose job exposes him or her to silica (silicosis), asbestos (asbestososis), or iron filings (siderosis). In addition, dust from wood, cotton, talc, cereal grains coffee, pesticides, drug or enzyme powders, or fiberglass may cause emphysema. People who use their lungs in their work are also susceptible (such as trumpet players and glass blowers). Any worker who is exposed to abnormal levels of dust, fumes, smoke, gases, vapors, or mists over a long period of time may be at risk for emphysema.

Sufferers typically complain that they "can't get enough air" as stale air builds up inside the lungs and the patient becomes starved of oxygen. Coughing, wheezing, and chronic mucous production are other common symptoms.

Diagnosis

A diagnosis of emphysema will not be made on the basis of the above symptoms alone. A detailed medical history will be taken along with x rays and pathology examinations. Peak flow tests will also be conducted.

Treatment

Damage to the lungs as a result of emphysema cannot be reversed, so preventative measures to limit its progression are essential. The following measures and treatments are regarded as beneficial for emphysema sufferers.

Herbalism

Herbs can be beneficial in relieving the symptoms of emphysema, helping the body to ward off infection, and easing the asthmatic symptoms that often accompany emphysema.

Some of them are:

• Lobelia: This is a mild sedative, also having strong expectorant properties. It is widely used for chest complaints, including emphysema and bronchitis, and can help to cut an asthma attack short.
• Thyme: A tea made with thyme is recommended for overcoming shortness of breath. It is also a powerful antiseptic.
• Mullein: This is another traditional remedy for chest complaints. Boil two tablespoons of the dried leaves with a glass of milk and drink.
• Echinacea: Echinacea is a powerful immune system stimulant and will strengthen the body in general, warding off colds and infections.
• Lungwort: A member of the borage family, this herb is very healing for the lungs. It should be taken as an infusion.
• Black cohosh: This herb is an expectorant and astringent. It relieves coughing.
• Sage: This is one of the most useful of all herbs and is said to be good for whatever it is taken for. It is antiviral and bactericidal.
• Garlic: A very powerful anti-viral, garlic can be of real help to those trying to avoid infections and lung congestion.

Chinese Herbal Medicine

Qing Qi Hua Tan Wan (Pinellia expectorant pills) are the Chinese herbalists' treatment for chronic lung complaints, particularly bronchitis and asthma.

Juices for Emphysema

Herbalist Kitty Campion recommends the following juices for the treatment of emphysema: equal parts of carrot juice, parsnip juice, watercress juice and potato juice, or equal parts of orange juice and lemon juice, diluted half and half with a strong decoction of rosehip tea.

Aromatherapy

Aromatherapy involves massaging the patient with potent plant essential oils, which have been proven to enter the circulation through the skin. The constituents of the oils can have a powerful effect on a variety of illnesses, but since their beneficial qualities are also transported through the air, they are considered to be doubly beneficial to those who suffer from respiratory ailments.

Aromatherapy oils for respiratory disease:

• Canada balsam may alleviate respiratory symptoms and is an expectorant. It is also a bactericide and recommended for those suffering from chronic chest ailments.
• Tolu balsam is an excellent treatment for chest infections.
• Frankincense is good for infection and catarrhal discharge.
• Niaouli is a very strong antiseptic and beneficial for pulmonary trouble.
• Rose damascena is recommended for bronchial complaints, and also uplifts the spirits.
• Tea tree oil was recently discovered to be one of the most potent anti-viral, anti-bacterial and anti-fungal agents known to medicine. Therefore highly beneficial as a preventative measure against chest infection.

Acupuncture

This ancient Chinese system of holistic treatment works on the principal that illness is the result of blockage in the flow of life force. The practitioner aims to stimulate relevant meridians in the body, and so release trapped life force, returning bodily functions to normal. The treatment is virtually painless.

Treatment can be expected to improve blood circulation and the capacity of the body to restore itself. Research has indicated that acupuncture can produce changes in the electrical fields of body cells, promoting a return to the body's normal state. Consequently, few negative side effects are associated with acupuncture treatment.

Breathing Techniques

Very few people actually breathe correctly, and if lung function is not up to par, the difference between breathing fully and taking shallow ineffective, breaths can make a remarkable difference in the way a person feels and the way his or her body functions. Oxygen shortage in the body promotes disease, and ensuring that oxygen levels are kept up can avert disaster, even with the existence of lung-impairment. Improved breathing techniques can rid the body of free radicals, neutralize environmental toxins, and destroy many harmful microbes that cannot exist in an oxygen-rich environment. Without sufficient oxygen, the body cannot fully utilize nutrients from food, and bodily functions generally become less efficient. Every effort must be made to promote proper breathing, in order to offset the effects of reduced lung function.

In cases of emphysema, it is particularly important to ensure that the out-breath expels all of the previous inbreath. When exhalation is incomplete, wastes produced by breathing are not expelled from the body in the normal way, and residual volume, which is a common occurrence with progressive emphysema, may cause chest enlargement.

Homeopathy

Homeopathy is the treatment of illness according to a system of "like cures like" that stimulates the body to heal itself. While it could definitely contribute to the successful treatment of emphysema, Homeopathy requires a qualified practitioner, as the patient's condition must be accurately assessed in order that the correct remedy be prescribed. Even for the same disorder, no two patients will receive the same treatment.

Lifestyle

For lung dysfunction of any kind, it is vital to take steps to ensure that a person's lifestyle is not contributing to the problem. Pollution must be avoided at all costs, and steps should be taken to ensure that the living environment is free of chemical irritants. This may involve avoiding fragrances, as they can overburden damaged lungs. Some unscented products use a masking fragrance which only increases toxicity. Common household products, such as fabric softeners, bleach, scented detergents, and furniture polish, can harm the body and the environment.

It must be noted that pesticides, fungicides, herbicides, and fertilizers are all neurotoxins, (poisonous to the nervous system). Natural alternatives are obtainable for most household cleaning products. Personal care products can also cause damage, so only natural sources should be used. Chlorinated pools should be avoided.

Every effort should be made to obtain food that is organically grown, in order to avoid pesticides and chemicals. Processed foods should be avoided because they often contain chemicals, dyes, and preservatives, and because the food is stripped of most of its nutritional value. Notably, artificial sweeteners, particularly aspartame, break down into deadly poisons in the body.

Clothing should be all natural fibers, as permanent press and wrinkle-resistant clothes have often been treated with formaldehyde which does not wash out. For the same reasons, synthetic fiber bed coverings should also be avoided. All plastic products should be avoided as far as possible as they all have toxic elements. Windows should be open as often as possible to increase oxygen in the atmosphere. Some houseplants should be acquired, as they give off oxygen.

It is also very important to undertake some form of gentle, regular exercise as this can do much to improve symptoms. Suitable forms of exercise may be swimming, walking and gentle rebounding. If an emphysema patient is very weak, he or she could sit on a mini-trampoline while a helper does the strenuous bit; very real benefits will still be obtained in this way. Strenuous activities are not suitable for anyone with lung impairment.

Naturopathy

According to the principles of naturopathy, the body has the power to heal itself. Treatment should focus on providing the system with optimum nutrition so it can carry out all repairs necessary. This involves ensuring that all food that is eaten is of the highest quality.

Naturopaths advocate dietary supplements to assist with this process, and certain dietary supplements can be very valuable in arresting the progress of Emphysema. Trials have been conducted involving treating emphysema patients with vitamin A, which is known to play an important role in healthy body tissue. Vitamin E can also be helpful, and vitamin C should always be taken, as it is a catalyst for other nutrients. For best results, it is advised to consult a practitioner.

Allopathic Treatment

Prior to any other treatment, it is essential that emphysema sufferers who smoke take steps to give up the habit. Otherwise, damage to the lungs will continue to go unchecked and other measures will be very limited in their success. Apart from lifestyle changes, physicians generally recommend avoidance of infection, and antibiotics may be prescribed as a preventative measure.

A physician may also prescribe bronchodilator medicines, which are usually prescribed for asthma patients, if there is any obstruction of the airways. For the same reason, anti-inflammatories may also be prescribed.

Chest physiotherapy, breathing exercises, and a program of physical exercise (collectively referred to as pulmonary rehabilitation) are considered beneficial to all emphysema patients, regardless of the degree of impairment. Supplementary oxygen may be required at some stage.

In extreme cases, lung volume reduction surgery may be recommended. If successful, this can eliminate the need for supplemental oxygen and improves breathing function. In this procedure, the damaged parts of the lung are removed in order to allow healthy lung tissue to expand. Careful evaluation of patients is carried out prior to this procedure. A final resort is lung transplant surgery. Because of the relatively large risk involved, this is carried out in only a small minority of patients.

Expected Results

It is generally accepted that emphysema is incurable. Physicians and alternative medicine practitioners assert that they can relieve sufferers greatly from symptoms and halt the progress of the disease with appropriate management and preventative measures.

Prevention

Any person who feels that his/her work conditions are likely to be a possible cause of emphysema should take steps to protect him/herself. A respirator should be worn, at least until work conditions can be improved. Several steps may be taken to improve conditions, primary of which should be to improve ventilation.

Early diagnosis is vital to the successful management of emphysema. If preventative and therapeutic measures are taken at the early onset of symptoms, damage can be restricted and the outlook can be positive. At all times, care should be taken to eliminate sources of pollution or chemical irritants from the environment, both in the home and elsewhere. The first step in overcoming emphysema for any patient should be to remove the cause, whether working conditions, polluted atmosphere or smoking.

Resources

Books

Ryman, Daniele. Aromatherapy London: Piatkus Books, 1999.

Treacher, Sylvia. Practical Homeopathy UK: Parragon Books, 2000.

Organizations

The National Emphysema Foundation

Other

"Progress in Emphysema Research. <"http://www.lrri.org/gobmasso.html> (January 17, 2001).

[Article by: Patricia Skinner]

Emphysema is a lung disease that, along with chronic bronchitis, represents a type of chronic obstructive pulmonary disease (COPD). Medical scientists have defined emphysema as "a condition of the lung characterized by abnormal, permanent enlargement of airspaces distal to the terminal bronchioles, accompanied by the destruction of their walls, and without obvious fibrosis" (Snider 1985).

COPD is the fourth leading cause of death in the United States, accounting for about 113,000 deaths annually. About 14 million Americans have symptoms of COPD. Among these, 1.65 million have emphysema. Millions more likely have undiagnosed or incipient COPD. The prevalence of COPD peaks in the sixty-five to seventy-four age range, and men are affected more than women.

Pathologists recognize three major types of emphysema: localized (distal acinar, paraseptal), centrilobular (centriacinar), and panlobular (panacinar). Centrilobular emphysema, the most common of the three, is usually caused by cigarette smoking. Cigarette smoke is thought to cause chronic inflammation in the walls of the air sacs (alveoli) of the lung, leading to an imbalance between destructive proteases and protective protease inhibitors. The proteases, such as elastase, gradually destroy the structural proteins (elastin, collagen) in the alveolar walls. Substantial variation in individual susceptibility to cigarette smoke exists, as only about one in seven cigarette smokers develops symptoms of COPD. Other than cigarette smoking, the only condition clearly linked to emphysema is a hereditary disorder called alpha₁-antitrypsin deficiency (AAT). This rare condition, which is found in less than one percent of patients with COPD, occurs because the blood level of a glycoprotein (protease inhibitor) is not sufficient to counteract the activity of the proteases. Coal miners and workers chronically exposed to cadmium fumes are at risk to develop emphysema. The effects of other occupational agents, air pollution, and familial factors in the pathogenesis of emphysema are not clear.

Destruction of alveolar walls in emphysema reduces the lung's elasticity, which results in obstruction to airflow in small airways, trapping air in the lung. Other pathophysiologic findings in emphysema include increased lung compliance, elevation of the pressure in the pulmonary arteries (pulmonary hypertension), and abnormal matching of air flow and blood flow (ventilation/perfusion imbalance), which causes hypoxemia (low oxygen level in the blood).

Patients with emphysema suffer from shortness of breath (dyspnea), which typically appears between the ages of fifty and sixty. Initially, the dyspnea is noted only with heavy exertion, but it progresses over time to a persistent, daily symptom that may eventually limit simple activities and even be present at rest. If the patient also has chronic bronchitis, daily cough and sputum production are present. Physical examination in emphysema reveals chest hyperinflation (overdistention) and reduced breath sounds on auscultation (listening to breathing noises with a stethoscope). In severe cases, there may be signs of respiratory failure and failure of the right side of the heart (cor pulmonale).

The clinical diagnosis of emphysema is suggested by the presence of a risk factor for emphysema (smoking and/or AAT), the clinical findings described above, the absence of alternative diagnoses to explain these findings (e.g., bronchial asthma, bronchiectasis, and central airways obstructive diseases), and evidence of airflow obstruction on spirometry (pulmonary function testing). Airflow obstruction in emphysema is usually irreversible, meaning there is no improvement in the obstruction after inhaling a bronchodilator drug. Specialized pulmonary tests may demonstrate air trapping and reduction in the gas-transfer ability of the lung. The chest radiograph in mild emphysema may be normal, but in severe cases there is hyperinflation. Sometimes large air sacs called bullae are seen. Computed tomographic imaging may confirm lung destruction, bullae, and hyperinflation. Arterial blood-gas analysis and transcutaneous measurement of oxyhemoglobin saturation (oximetry) reveal hypoxemia in advanced emphysema.

Emphysema is treated with a broad-based approach that includes elimination of cigarette smoking, immunization against influenza virus and Streptococcus pneumoniae infection, exercise, maintenance of a healthy lifestyle, and the use of bronchodilator medications (e.g., ipratropium bromide and albuterol). Supplemental oxygen is prescribed if hypoxemia is present. Continuous long-term oxygen therapy improves survival in COPD patients with hypoxemia. Anti-inflammatory drugs such as corticosteroids are helpful in a small percent of emphysema patients. COPD exacerbations, with increasing dyspnea, cough, and sputum production, are usually treated with intensification of the bronchodilator regimen, antibiotics, supplemental oxygen, and in some cases corticosteroids. Hospitalization may be necessary, and in severe cases insertion of a breathing tube into the airway (endotracheal intubation) and mechanical ventilation are necessary. Debilitated COPD patients may benefit from comprehensive outpatient rehabilitation. Rarely, patients with advanced emphysema are treated surgically (removal of large bullae, volume reduction surgery, or lung transplantation).

With the exception of AAT, emphysema is a preventable disease. Smoking abstinence remains the best hope for reducing the morbidity and mortality associated with emphysema. Early detection of airflow limitation in young cigarette smokers may provide a strong stimulus to quit smoking. This is important because smoking cessation is known to slow the rate of decline in lung function in middle-aged smokers with mild COPD.

Survival in patients with COPD is determined by multiple factors, including age, gender, lung function, and levels of oxygen and carbon dioxide in the blood. The prognosis is worse when the airflow obstruction is irreversible. COPD patients with severe obstruction, as defined by spirometry, have a median survival of about four to five years, but there is substantial variability. Death in emphysema patients is usually a result of pneumonia, lung cancer, heart disease, or respiratory failure.

(SEE ALSO: Asthma; Chronic Respiratory Diseases; Pulmonary Function; Smoking Behavior; Smoking Cessation; Tobacco Control)

Bibliography

American Thoracic Society (1995). "Standards for the Diagnosis and Care of Patients with Chronic Obstructive Pulmonary Disease." American Journal Respiratory Critical Care Medicine 152:S77–S120.

Anthonisen, N. R.; Connett, J. E.; Kiley, J. P.; Altose, M. D.; Bailey, W.C.; Buist, A. S.; Conway, W. A. Jr.; Enright, P. L.; Kanner, R. E.; O'Hara, P.; Owens, G. R.; Scanlon, P. D.; Tashkin, D. P.; and Wise, R. A.(1994). "Effects of Smoking Intervention and the Use of an Inhaled Anticholinergic Bronchodilator on the Rate of Decline of FEV₁. The Lung Health Study." Journal of the American Medical Association 272(19): 1497–1505.

Celli, B., Benditt, J.; and Albert, R. K. (1999) "Chronic Obstructive Pulmonary Disease." In Comprehensive Respiratory Medicine, eds. R. Albert, S. Spiro, and J. Jett, St. Louis, MO: Mosby.

Snider, G. L.; Kleinerman, J.; Thurlbeck, W. M.; and Bengali, Z. H. (1985). "The Definition of Emphysema. Report of a National Heart, Lung, and Blood Institute, Division of Lung Diseases Workshop." American Review of Respiratory Diseases 132:182–185.

— JOHN L. STAUFFER

For more information on emphysema, visit Britannica.com.

A degenerative disease, fairly common in the elderly, in which living tissue in the airway loses its elasticity so that air tends to remain trapped in the lungs. This effectively reduces breathing capacity and the ability to perform physical work. A sensibly prescribed programme of aerobic exercise can improve the work capacity of those with emphysema, but it will not cure the condition.

A chronic disease in which the tiny air sacs in the lungs become stretched and enlarged, so that they are less able to supply oxygen to the blood. Emphysema causes shortness of breath and painful coughing and can increase the likelihood of developing heart disease. Emphysema occurs most frequently in older men who have been heavy smokers.

Of the nature of or affected with emphysema.

• e. gangrene — see blackleg.

Emphysema
Classification and external resources
H&E (haematoxylin and eosin) stained lung tissue sample from an end-stage emphysema patient. RBCs are red, nuclei are blue-purple, other cellular and extracellular material is pink, and air spaces are white.
ICD-10	J43.
ICD-9	492
DiseasesDB	4190
MedlinePlus	000136
eMedicine	med/654
MeSH	D011656

For the condition describing air trapped under the skin, see subcutaneous emphysema.

Emphysema (pronounced /ˌɛmfɨˈziːmə/) is a chronic obstructive pulmonary disease (COPD, as it is otherwise known, formerly termed a chronic obstructive lung disease). It is often caused by exposure to toxic chemicals, including long-term exposure to tobacco smoke.

Contents 1 Presentation 2 Classification 2.1 Congenital lobar emphysema 3 Pathophysiology 4 Pathogenesis 5 Associations 6 Prognosis and treatment 7 Notable cases 8 See also 9 Footnotes

Presentation

Emphysema is characterized by loss of elasticity (increased pulmonary compliance) of the lung tissue caused by destruction of structures feeding the alveoli, owing to the action of alpha 1 antitrypsin deficiency. This causes the small airways to collapse during forced exhalation, as alveolar collapsibility has decreased. As a result, airflow is impeded and air becomes trapped in the lungs, in the same way as other obstructive lung diseases. Symptoms include shortness of breath on exertion, and an expanded chest. However, the constriction of air passages isn't always immediately deadly, and treatment is available.

Signs of emphysema include pursed-lipped breathing, central cyanosis and finger clubbing. The chest has hyper resonant percussion notes, particularly just above the liver, and a difficult to palpate apex beat, both due to hyperinflation. There may be decreased breath sounds and audible expiratory wheeze. In advanced disease, there are signs of fluid overload such as pitting peripheral edema. The face has a ruddy complexion if there is a secondary polycythemia. Sufferers who retain carbon dioxide have asterixis (metabolic flap) at the wrist.

Classification

Emphysema can be classified into primary and secondary. However, it is more commonly classified by location.

Emphysema can be subdivided into panacinary and centroacinary (or panacinar and centriacinar,^[1] or centrilobular and panlobular).^[2]

• Panacinary (or panlobular) emphysema is related to the destruction of alveoli, because of an inflammation or deficiency of alpha 1-antitrypsin. It is found more in young adults who do not have chronic bronchitis.
• Centroacinary (or centrilobular) emphysema is due to destruction of terminal bronchioli muchosis, due to chronic bronchitis. This is found mostly in elderly people with a long history of smoking or extreme cases of passive smoking.

Other types include distal acinar and irregular.^[1]

A special type is congenital lobar emphysema (CLE).

Congenital lobar emphysema

CLE is 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. ^[3]

There may be congenital extrinsic compression, commonly by an abnormally large pulmonary artery. This causes malformation of bronchial cartilage, making them soft and collapsible.^[3]

CLE is potentially reversible, yet possibly life-threatening, causing respiratory distress in the neonate.^[3]

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 deformation of the lung architecture.^[4] These deformations result in a large decrease of alveoli surface area used for gas exchange. To accommodate the decreased surface area, thoracic cage expansion (barrel chest) and diaphragm contraction (flattening) take place. Expiration 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. A1AD allows inflammatory enzymes (such as elastase) to destroy 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 (1–2 weeks).

Pathogenesis

Severe emphysema

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. Perhaps the most interesting 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 0–3 years at most.

Associations

Emphysema is commonly associated with bronchitis and chronic bronchitis. Since it is difficult to delineate "pure" cases of emphysema or chronic bronchitis, they are generally grouped together as chronic obstructive pulmonary disease (COPD).

See above for alpha 1-antitrypsin deficiency. Severe cases of A1AD may also develop cirrhosis of the liver, where the accumulated A1AT leads to a fibrotic reaction.

Prognosis and treatment

Emphysema is an irreversible degenerative condition. 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. Patients with emphysema and chronic bronchitis can do more for themselves than patients with any other disabling disease.

Emphysema is also treated by supporting the breathing with anticholinergics, bronchodilators, steroid medication (inhaled or oral), 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. Transplants also require the patient to take an anti-rejection drug regimen which suppresses the immune system, and so can lead to microbial infection of the patient. Patients who think they may have contracted the disease are recommended to seek medical attention as soon as possible.

A study published by the European Respiratory Journal suggests that tretinoin (an anti-acne drug commercially available as Retin-A) derived from vitamin A can reverse the effects of emphysema in mice by returning elasticity (and regenerating lung tissue through gene mediation) to the alveoli.^[5][6] While vitamin A consumption is not known to be an effective treatment or prevention for the disease, this research could in the future lead to a cure. A follow-up study done in 2006 found inconclusive results ("no definitive clinical benefits") using Vitamin A (retinoic acid) in treatment of emphysema in humans and stated that further research is needed to reach conclusions on this treatment.^[7]

Notable cases

Notable cases of emphysema have included Ava Gardner (67),^[8] Spencer Tracy (67),^[9] Leonard Bernstein (72), Eddie Dean (91),^[10] Dean Martin (78), Samuel Beckett (83), Johnny Carson (79), Al Capp (70), T. S. Eliot (76), Tallulah Bankhead (66), Dick York (63), James Franciscus (57), R. J. Reynolds (67), R. J. Reynolds Jr. (58), R. J. Reynolds, III (59),^[11] Don Imus,^[12] Ike Turner (76), Amy Winehouse, Elizabeth Dawn, Jerry Reed and Jessica Caroline Lunn. (71) ^[13]. See also Deaths from Emphysema.

See also

• Subcutaneous emphysema

Footnotes

1. ^ ^{a b} "Emphysema". http://www.meddean.luc.edu/Lumen/MedEd/MEDICINE/PULMONAR/pathms/mpath6.htm. Retrieved on 2008-11-20. 
2. ^ Anderson AE, Foraker AG (September 1973). "Centrilobular emphysema and panlobular emphysema: two different diseases". Thorax 28 (5): 547–50. doi:10.1136/thx.28.5.547. PMID 4784376. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pubmed&pubmedid=4784376. 
3. ^ ^{a b c} eMedicine Specialties > Radiology > Pediatrics --> Congenital Lobar Emphysema Author: Beverly P Wood, MD, MS, PhD, University of Southern California. Updated: Dec 1, 2008
4. ^ "SURGICAL PHYSIOPATHOLOGY OF EMPHYSEMA AND LUNG VOLUME REDUCTION". http://www.fondazionecarrel.org/carrel/thorac/files/enphys/new/emphysema1.html. 
5. ^ Mao J, Goldin J, Dermand J, Ibrahim G, Brown M, Emerick A, McNitt-Gray M, Gjertson D, Estrada F, Tashkin D, Roth M (01 Mar 2002). "A pilot study of all-trans-retinoic acid for the treatment of human emphysema". Am J Respir Crit Care Med 165 (5): 718–23. PMID 11874821. http://ajrccm.atsjournals.org/cgi/content/full/165/5/718. 
6. ^ "Vitamin may cure smoking disease". BBC News. December 22, 2003. http://news.bbc.co.uk/2/hi/health/3329103.stm. Retrieved on 2006-11-18. 
7. ^ Roth M, Connett J, D'Armiento J, Foronjy R, Friedman P, Goldin J, Louis T, Mao J, Muindi J, O'Connor G, Ramsdell J, Ries A, Scharf S, Schluger N, Sciurba F, Skeans M, Walter R, Wendt C, Wise R (2006). "Feasibility of retinoids for the treatment of emphysema study". Chest 130 (5): 1334–45. doi:10.1378/chest.130.5.1334. PMID 17099008. http://www.chestjournal.org/cgi/content/full/130/5/1334. 
8. ^ "Ava Gardner's Bio on Wiki"
9. ^ "Spencer Tracy bio at Hollywood.com"
10. ^ "Eddie Dean Obituary". Allbusiness.com. http://www.allbusiness.com/retail-trade/miscellaneous-retail-retail-stores-not/4625955-1.html. 
11. ^ "Death from Smoking in the R. J. Reynolds Family by Patrick Reynolds"
12. ^ "Don Imus's Last Stand: Politics & Power"
13. ^ "Amy Winehouse rushed to hospital"

v • d • e Pathology of respiratory system (J, 460-519), respiratory diseases Upper RT (including URTIs, Common cold) Head sinuses: Sinusitis nose: Rhinitis (Vasomotor rhinitis, Atrophic rhinitis, Hay fever) · Nasal polyp · Deviated septum · Rhinorrhea tonsil: Tonsillitis · Adenoid hypertrophy · Peritonsillar abscess Neck pharynx: Pharyngitis (Strep throat) larynx: Laryngitis · Croup · Laryngospasm · vocal folds: Vocal fold nodule · epiglottis: Epiglottitis trachea: Tracheitis · Tracheal stenosis Lower RT/lung disease (including LRTIs) Bronchial/ obstructive acute: Acute bronchitis chronic: COPD (Chronic bronchitis, Emphysema, Diffuse panbronchiolitis) · Asthma (Status asthmaticus) · Bronchiectasis unspecified: Bronchitis · Bronchiolitis (Bronchiolitis obliterans) Interstitial/ restrictive External agents/ occupational lung disease Pneumoconiosis (Asbestosis, Baritosis, Bauxite fibrosis, Berylliosis, Caplan's syndrome, Chalicosis, Coalworker's pneumoconiosis, Siderosis, Silicosis, Byssinosis) Hypersensitivity pneumonitis (Bagassosis, Bird fancier's lung, Farmer's lung) Other ARDS · Pulmonary edema · Löffler's syndrome/Eosinophilic pneumonia · Respiratory hypersensitivity (Allergic bronchopulmonary aspergillosis) Hamman-Rich syndrome · Idiopathic pulmonary fibrosis · Sarcoidosis Obstructive or restrictive Pneumonia/ pneumonitis By pathogen Viral · Bacterial (Pneumococcal, Klebsiella) / Atypical bacterial (Mycoplasma, Legionnaires' disease, Chlamydiae) · Fungal (Pneumocystis) · Parasitic · noninfectious (Chemical/Mendelson's syndrome, Aspiration/Lipid) By vector/route Community-acquired · Healthcare-associated · Hospital-acquired By distribution Broncho- · Lobar IIP UIP · DIP · BOOP-COP · NSIP · RB Other Atelectasis · circulatory (Pulmonary hypertension, Pulmonary embolism) Pleural cavity/ mediastinum Suppuration Empyema/pyothorax · Lung abscess Pleural disease Pleuritis/pleurisy Pneumothorax/Hemopneumothorax (Tension pneumothorax) Pleural effusion: Hemothorax · Hydrothorax · Chylothorax Fibrothorax Mediastinal disease Mediastinitis · Mediastinal emphysema Other/general Respiratory failure · Influenza · SARS · Idiopathic pulmonary haemosiderosis · Pulmonary alveolar proteinosis see also congenital, neoplasia

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Common misspelling(s) of emphysema

• emphysyma

Dansk (Danish)
n. - emfysem

Nederlands (Dutch)
emfyseem (zwellen van lichamelijk weefsel door luchtophoping)

Français (French)
n. - emphysème

Deutsch (German)
n. - (Med.) Emphysem (Luftansammlung im Gewebe)

Ελληνική (Greek)
n. - (παθολ.) εμφύσημα

Italiano (Italian)
enfisema

Português (Portuguese)
n. - enfisema (m) (Med.)

Русский (Russian)
эмфизема

Español (Spanish)
n. - enfisema

Svenska (Swedish)
n. - emfysem

中文（简体）(Chinese (Simplified))
气肿, 肺气肿

中文（繁體）(Chinese (Traditional))
n. - 氣腫, 肺氣腫

한국어 (Korean)
n. - 기종, 폐기종

日本語 (Japanese)
n. - 気腫

العربيه (Arabic)
‏(الاسم) انتفاخ‏

עברית (Hebrew)
n. - ‮התנפחות הריאות, נפחת, התנפחות כתוצאה ממציאות אוויר ברקמות-החיבור של הגוף‬

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