Emphysema
Chronic obstructive pulmonary disease
COPD; Chronic obstructive airways disease; Chronic obstructive lung disease; Chronic bronchitis; Emphysema; Chronic obstructive pulmonary disease (COPD) is one of the most common lung diseases. It makes it difficult to breathe. There are two main forms of COPD:
- Chronic bronchitis, defined by a long-term cough with mucus
- Emphysema, defined by destruction of the lungs over time
- Most people with COPD have a combination of both conditions.
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 smoking or long-term exposure to air pollution. The term means swelling
Emphysema can be classified into primary and secondary. However, it is more commonly classified by location into panacinary and centroacinary (or panacinar and centriacinar, or centrilobular and panlobular).
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.
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. Other types include distal acinar and irregular. A special type is congenital lobar emphysema.
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.
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 person’s 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.
Causes, incidence, and risk factors:
Smoking is the leading cause of COPD. The more a person smokes, the more likely that person will develop COPD although some people smoke for years and never get COPD. In rare cases, nonsmokers who lack a protein called alpha-1 antitrypsin can develop emphysema.
Other risk factors for COPD are:
1. Exposure to certain gases or fumes in the workplace
2. Exposure to heavy amounts of secondhand smoke and pollution
3. Frequent use of cooking gas without proper ventilation
Symptoms:
- Cough with mucus
- Shortness of breath (dyspnea) that gets worse with mild activity
- Fatigue
- Frequent respiratory infections
- Wheezing
- Since the symptoms of COPD develop slowly, some people may be unaware that they are sick.
Signs and tests:
The best test for COPD is a simple lung function test called spirometry. This involves blowing out as hard as one can into a small machine that tests lung capacity. The test can be interpreted immediately and does not involve exercising, drawing blood, or exposure to radiation.
Using a stethoscope to listen to the lungs can also be helpful, although sometimes the lungs sound normal even when COPD is present.
Pictures of the lungs (such as X-rays and CT scans) can be helpful but sometimes look normal even when a person has COPD.
Sometimes it is necessary to do a blood test (call a “blood gas”) to measure the amounts of oxygen and carbon dioxide in the blood
Treatment:
There is no cure for COPD. However, there are many things you can do to relieve symptoms and keep the disease from getting worse.
Persons with COPD must stop smoking. This is the best way to slow down the lung damage.
Medications used to treat COPD include:
Inhalers (bronchodilators) to open the airways, such as ipratropium (Atrovent), tiotropium (Spiriva), salmeterol (Serevent), or formoterol (Foradil)
Inhaled steroids to reduce lung inflammation:
In severe cases or during flare-ups, you may need to receive steroids by mouth or through a vein (intravenously).
Antibiotics are prescribed during symptom flare-ups, because infections can make COPD worse.
Oxygen therapy at home may be needed if a person has a low level of oxygen in their blood. Pulmonary rehabilitation does not cure the lung disease, but it can teach you to breathe in a different way so you can stay active. Exercise programs such as pulmonary rehabilitation are also important to help maintain muscle strength in the legs so less demand is placed on the lungs when walking. These programs also teach people how to use their medicines most effectively.
Things you can do to make it easier for yourself around the home include:
- Avoiding very cold air
- Making sure no one smokes in your home
- Reducing air pollution by eliminating fireplace smoke and other irritants
- Eat a healthy diet with fish, poultry, or lean meat, as well as fruits and vegetables. If it is hard to keep your weight up, talk to a doctor or dietitian about getting foods with more calories.
Surgical treatments may include:
- Surgery to remove parts of the diseased lung, for some patients with emphysema
- Lung transplant for severe cases
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 twenty 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 seven percent 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. Patients who think they may have contracted the disease are recommended to seek medical attention as soon as possible.
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.
Expectations (prognosis):
This condition is a long-term (chronic) illness. The disease will get worse more quickly if one continues to smoke.
Patients with severe COPD will be short of breath with most activities and will be admitted to the hospital more often. These patients should talk with their doctor about the use of breathing machines and end-of-life care.
Complications:
- Irregular heartbeat (arrhythmias)
- Need for breathing machine and oxygen therapy
- Right-sided heart failure or cor pulmonale (heart swelling and heart failure due to chronic lung disease)
- Pneumonia
- Pneumothorax
- Severe weight loss and malnutrition
Calling your health care provider:
Go to the emergency room or call the local emergency number (such as 911) if you have a rapid increase in shortness of breath
Prevention:
Not smoking prevents most COPD. Ask your doctor or health care provider about quit-smoking programs. Medicines are also available to help kick the smoking habit, and the medicines are most effective if a person is motivated to quit.
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), does not 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 0–3 years at most.
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References
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