Chronic Obstructive Pulmonary Disease

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Synonyms

COPD,

Overview

Chronic obstructive pulmonary disease (COPD) is a chronic inflammatory lung disease that causes obstructed airflow from the lungs. Symptoms include breathing difficulty, cough, mucus (sputum) production and wheezing. It’s typically caused by long-term exposure to irritating gases or particulate matter, most often from cigarette smoke. People with COPD are at increased risk of developing heart disease, lung cancer and a variety of other conditions.

Emphysema and chronic bronchitis are the two most common conditions that contribute to COPD. These two conditions usually occur together and can vary in severity among individuals with COPD.

Chronic bronchitis is inflammation of the lining of the bronchial tubes, which carry air to and from the air sacs (alveoli) of the lungs. It’s characterized by daily cough and mucus (sputum) production.

Emphysema is a condition in which the alveoli at the end of the smallest air passages (bronchioles) of the lungs are destroyed as a result of damaging exposure to cigarette smoke and other irritating gases and particulate matter.

Although COPD is a progressive disease that gets worse over time, COPD is treatable. With proper management, most people with COPD can achieve good symptom control and quality of life, as well as reduced risk of other associated conditions.

Symptoms

COPD symptoms often don’t appear until significant lung damage has occurred, and they usually worsen over time, particularly if smoking exposure continues.

Signs and symptoms of COPD may include:

Shortness of breath, especially during physical activities

Wheezing

Chest tightness

A chronic cough that may produce mucus (sputum) that may be clear, white, yellow or greenish

Frequent respiratory infections

Lack of energy

Unintended weight loss (in later stages)

Swelling in ankles, feet or legs

People with COPD are also likely to experience episodes called exacerbations, during which their symptoms become worse than the usual day-to-day variation and persist for at least several days.

Causes

The main cause of COPD in developed countries is tobacco smoking. In the developing world, COPD often occurs in people exposed to fumes from burning fuel for cooking and heating in poorly ventilated homes.

Only some chronic smokers develop clinically apparent COPD, although many smokers with long smoking histories may develop reduced lung function. Some smokers develop less common lung conditions. They may be misdiagnosed as having COPD until a more thorough evaluation is performed.

Causes of airway obstruction include:

  • Emphysema. This lung disease causes destruction of the fragile walls and elastic fibers of the alveoli. Small airways collapse when you exhale, impairing airflow out of your lungs.
  • Chronic bronchitis. In this condition, your bronchial tubes become inflamed and narrowed and your lungs produce more mucus, which can further block the narrowed tubes. You develop a chronic cough trying to clear your airways.

In the vast majority of people with COPD, the lung damage that leads to COPD is caused by long-term cigarette smoking. But there are likely other factors at play in the development of COPD, such as a genetic susceptibility to the disease, because not all smokers develop COPD.

Other irritants can cause COPD, including cigar smoke, secondhand smoke, pipe smoke, air pollution, and workplace exposure to dust, smoke or fumes.

In about 1% of people with COPD, the disease results from a genetic disorder that causes low levels of a protein called alpha-1-antitrypsin (AAt). AAt is made in the liver and secreted into the bloodstream to help protect the lungs. Alpha-1-antitrypsin deficiency can cause liver disease, lung disease or both.

For adults with COPD related to AAt deficiency, treatment options include those used for people with more-common types of COPD. In addition, some people can be treated by replacing the missing AAt protein, which may prevent further damage to the lungs.

Prevention

Unlike some diseases, COPD typically has a clear cause and a clear path of prevention, and there are ways to slow the progression of the disease. The majority of cases are directly related to cigarette smoking, and the best way to prevent COPD is to never smoke — or to stop smoking now.

If you’re a longtime smoker, these simple statements may not seem so simple, especially if you’ve tried quitting — once, twice or many times before. But keep trying to quit. It’s critical to find a tobacco cessation program that can help you quit for good. It’s your best chance for reducing damage to your lungs.

Occupational exposure to chemical fumes and dusts is another risk factor for COPD. If you work with these types of lung irritants, talk to your supervisor about the best ways to protect yourself, such as using respiratory protective equipment.

Here are some steps you can take to help prevent complications associated with COPD:

Quit smoking to help reduce your risk of heart disease and lung cancer.

Get an annual flu vaccination and regular vaccination against pneumococcal pneumonia to reduce your risk of or prevent some infections.

Talk to your doctor if you feel sad or helpless or think that you may be experiencing depression.

Diagnosis

COPD is commonly misdiagnosed. Many people who have COPD may not be diagnosed until the disease is advanced.

To diagnose your condition, your doctor will review your signs and symptoms, discuss your family and medical history, and discuss any exposure you’ve had to lung irritants — especially cigarette smoke. Your doctor may order several tests to diagnose your condition.

Tests may include:

Lung (pulmonary) function tests. These tests measure the amount of air you can inhale and exhale, and whether your lungs deliver enough oxygen to your blood. During the most common test, called spirometry, you blow into a large tube connected to a small machine to measure how much air your lungs can hold and how fast you can blow the air out of your lungs. Other tests include measurement of lung volumes and diffusing capacity, six-minute walk test, and pulse oximetry.

Chest X-ray. A chest X-ray can show emphysema, one of the main causes of COPD. An X-ray can also rule out other lung problems or heart failure.

CT scan. A CT scan of your lungs can help detect emphysema and help determine if you might benefit from surgery for COPD. CT scans can also be used to screen for lung cancer.

Arterial blood gas analysis. This blood test measures how well your lungs are bringing oxygen into your blood and removing carbon dioxide.

Laboratory tests. Lab tests aren’t used to diagnose COPD, but they may be used to determine the cause of your symptoms or rule out other conditions. For example, lab tests may be used to determine if you have the genetic disorder alpha-1-antitrypsin deficiency, which may be the cause of COPD in some people. This test may be done if you have a family history of COPD and develop COPD at a young age

Treatment

Many people with COPD have mild forms of the disease for which little therapy is needed other than smoking cessation. Even for more advanced stages of disease, effective therapy is available that can control symptoms, slow progression, reduce your risk of complications and exacerbations, and improve your ability to lead an active life.

Quitting smoking

The most essential step in any treatment plan for COPD is to quit all smoking. Stopping smoking can keep COPD from getting worse and reducing your ability to breathe. But quitting smoking isn’t easy. And this task may seem particularly daunting if you’ve tried to quit and have been unsuccessful.

Talk to your doctor about nicotine replacement products and medications that might help, as well as how to handle relapses. Your doctor may also recommend a support group for people who want to quit smoking. Also, avoid secondhand smoke exposure whenever possible.

Medications

Several kinds of medications are used to treat the symptoms and complications of COPD. You may take some medications on a regular basis and others as needed.

Bronchodilators

Bronchodilators are medications that usually come in inhalers — they relax the muscles around your airways. This can help relieve coughing and shortness of breath and make breathing easier. Depending on the severity of your disease, you may need a short-acting bronchodilator before activities, a long-acting bronchodilator that you use every day or both.

Examples of short-acting bronchodilators include:

  • Albuterol (ProAir HFA, Ventolin HFA, others)
  • Ipratropium (Atrovent HFA)
  • Levalbuterol (Xopenex)

Examples of long-acting bronchodilators include:

  • Aclidinium (Tudorza Pressair)
  • Arformoterol (Brovana)
  • Formoterol (Perforomist)
  • Indacaterol (Arcapta Neoinhaler)
  • Tiotropium (Spiriva)
  • Salmeterol (Serevent)
  • Umeclidinium (Incruse Ellipta)

Inhaled steroids

Inhaled corticosteroid medications can reduce airway inflammation and help prevent exacerbations. Side effects may include bruising, oral infections and hoarseness. These medications are useful for people with frequent exacerbations of COPD. Examples of inhaled steroids include:

  • Fluticasone (Flovent HFA)
  • Budesonide (Pulmicort Flexhaler)

Combination inhalers

Some medications combine bronchodilators and inhaled steroids. Examples of these combination inhalers include:

  • Fluticasone and vilanterol (Breo Ellipta)
  • Fluticasone, umeclidinium and vilanterol (Trelegy Ellipta)
  • Formoterol and budesonide (Symbicort)
  • Salmeterol and fluticasone (Advair HFA, AirDuo Digihaler, others)

Combination inhalers that include more than one type of bronchodilator also are available. Examples of these include:

  • Aclidinium and formoterol (Duaklir Pressair)
  • Albuterol and ipratropium (Combivent Respimat)
  • Formoterol and glycopyrrolate (Bevespi Aerosphere)
  • Glycopyrrolate and indacaterol (Utibron)
  • Olodaterol and tiotropium (Stiolto Respimat)
  • Umeclidinium and vilanterol (Anoro Ellipta)

Oral steroids

For people who experience periods when their COPD becomes more severe, called moderate or severe acute exacerbation, short courses (for example, five days) of oral corticosteroids may prevent further worsening of COPD. However, long-term use of these medications can have serious side effects, such as weight gain, diabetes, osteoporosis, cataracts and an increased risk of infection.

Phosphodiesterase-4 inhibitors

A medication approved for people with severe COPD and symptoms of chronic bronchitis is roflumilast (Daliresp), a phosphodiesterase-4 inhibitor. This drug decreases airway inflammation and relaxes the airways. Common side effects include diarrhea and weight loss.

Theophylline

When other treatment has been ineffective or if cost is a factor, theophylline (Elixophyllin, Theo-24, Theochron), a less expensive medication, may help improve breathing and prevent episodes of worsening COPD. Side effects are dose related and may include nausea, headache, fast heartbeat and tremor, so tests are used to monitor blood levels of the medication.

Antibiotics

Respiratory infections, such as acute bronchitis, pneumonia and influenza, can aggravate COPD symptoms. Antibiotics help treat episodes of worsening COPD, but they aren’t generally recommended for prevention. Some studies show that certain antibiotics, such as azithromycin (Zithromax), prevent episodes of worsening COPD, but side effects and antibiotic resistance may limit their use.

Lung therapies

Doctors often use these additional therapies for people with moderate or severe COPD:

  • Oxygen therapy. If there isn’t enough oxygen in your blood, you may need supplemental oxygen. There are several devices that deliver oxygen to your lungs, including lightweight, portable units that you can take with you to run errands and get around town.

    Some people with COPD use oxygen only during activities or while sleeping. Others use oxygen all the time. Oxygen therapy can improve quality of life and is the only COPD therapy proved to extend life. Talk to your doctor about your needs and options.

  • Pulmonary rehabilitation program. These programs generally combine education, exercise training, nutrition advice and counseling. You’ll work with a variety of specialists, who can tailor your rehabilitation program to meet your needs.

    Pulmonary rehabilitation after episodes of worsening COPD may reduce readmission to the hospital, increase your ability to participate in everyday activities and improve your quality of life. Talk to your doctor about referral to a program.

In-home noninvasive ventilation therapy

Evidence supports in-hospital use of breathing devices such as bilevel positive airway pressure (BiPAP), but some research now supports the benefit of its use at home. A noninvasive ventilation therapy machine with a mask helps to improve breathing and decrease retention of carbon dioxide (hypercapnia) that may lead to acute respiratory failure and hospitalization. More research is needed to determine the best ways to use this therapy.

Managing exacerbations

Even with ongoing treatment, you may experience times when symptoms become worse for days or weeks. This is called an acute exacerbation, and it may lead to lung failure if you don’t receive prompt treatment.

Exacerbations may be caused by a respiratory infection, air pollution or other triggers of inflammation. Whatever the cause, it’s important to seek prompt medical help if you notice a sustained increase in coughing or a change in your mucus, or if you have a harder time breathing.

When exacerbations occur, you may need additional medications (such as antibiotics, steroids or both), supplemental oxygen or treatment in the hospital. Once symptoms improve, your doctor can talk with you about measures to prevent future exacerbations, such as quitting smoking; taking inhaled steroids, long-acting bronchodilators or other medications; getting your annual flu vaccine; and avoiding air pollution whenever possible.

Surgery

Surgery is an option for some people with some forms of severe emphysema who aren’t helped sufficiently by medications alone. Surgical options include:

  • Lung volume reduction surgery. In this surgery, your surgeon removes small wedges of damaged lung tissue from the upper lungs. This creates extra space in your chest cavity so that the remaining healthier lung tissue can expand and the diaphragm can work more efficiently. In some people, this surgery can improve quality of life and prolong survival.

    Endoscopic lung volume reduction — a minimally invasive procedure — has recently been approved by the U.S. Food and Drug Administration to treat people with COPD. A tiny one-way endobronchial valve is placed in the lung, allowing the most damaged lobe to shrink so that the healthier part of the lung has more space to expand and function.

  • Lung transplant. Lung transplantation may be an option for certain people who meet specific criteria. Transplantation can improve your ability to breathe and to be active. However, it’s a major operation that has significant risks, such as organ rejection, and you’ll need to take lifelong immune-suppressing medications.
  • Bullectomy. Large air spaces (bullae) form in the lungs when the walls of the air sacs (alveoli) are destroyed. These bullae can become very large and cause breathing problems. In a bullectomy, doctors remove bullae from the lungs to help improve air flow.