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Bronchiectasis

Bronchiectasis: Causes, Symptoms, Diagnosis, Treatment, and More

Featured Expert: Neil Schachter, MD

When you breathe, the air you inhale travels to your lungs through a branching system of flexible tubes known as airways (or bronchi). But a condition called bronchiectasis can interfere with this system, reducing airflow, rendering the airways more susceptible to inflammation and infections, and causing a chronic cough.

Bronchiectasis affects an estimated 350,000 to 500,000 Americans, and older people are among those most vulnerable to the condition. Fortunately, modern treatments and self-help strategies can help mitigate many of the complications.

What Is Bronchiectasis?

The airways normally widen as you inhale and then resume their original shape as you exhale. This smooth transition during breathing is assisted by supporting tissues that surround the airways and includes spiral bands of muscle as well as elastic and connective tissue. These tissues allow the airways to expand uniformly as you inhale and to narrow without collapsing when you exhale.

“Bronchiectasis is characterized by damage to these underlying tissues,” says Mount Sinai pulmonologist E. Neil Schachter, MD. “The damage, when severe, cannot be reversed. It essentially makes the airways ‘floppy,’ and, as result, they become abnormally wide and may develop ballooned sections that resemble varicose veins. These changes make coughing less effective.”  

The airways also have surface lining cells, including mucus-producing cells and “ciliated cells,” so called because their surface is covered with tiny hair-like structures called cilia. “The mucus that is produced in the airways traps irritating particles and germs,” Dr. Schachter explains. “The tiny hair-like projections on ciliated cells sweep the material trapped in the mucus up the airway into the larger upstream airways, from which it can be coughed up or swallowed.” In bronchiectasis, this protective sequence fails. “Instead of being efficiently removed from the lung airways, mucus pools in the ballooned sections of the damaged airways,” Dr. Schachter says. “The excess mucus, which harbors bacteria and irritants, can cause recurrent lung infections that worsen the airway damage and cause lung function to further deteriorate over time.”

Bronchiectasis Symptoms

Some people with bronchiectasis experience few symptoms, particularly if the disease is mild and affects only limited sections of the lung. “In these cases, healthy lung tissue is able to compensate to an extent that masks breathing difficulties,” Dr. Schachter says. “When symptoms do occur, they tend to set in gradually, following a respiratory tract infection, and worsen during flare-ups.” Known as exacerbations, flare-ups can indicate that bronchiectasis is progressing to cause more severe tissue damage in the airways.

Bronchiectasis symptoms typically include:

  • A chronic cough that occurs every day and produces large amounts of thick, yellow or greenish mucus that occasionally may be tinged with blood from the damaged airways.
  • Shortness of breath, particularly with exertion.
  • Wheezing: a high-pitched sound that results from airflow restriction.
  • Chest pain due to persistent coughing.
  • Fatigue that stems from chronic low-grade infection and/or inflammation as well as difficult breathing and coughing.
  • Weight loss occurs because swallowing excess mucus may lead to loss of appetite. You also may simply lack the energy to prepare nourishing meals.

What Causes Bronchiectasis?

Bronchiectasis tends to occur in people who have airway damage due to poorly treated, incompletely treated, or untreated lung infections such as tuberculosis (the most common cause of bronchiectasis worldwide), pertussis (whooping cough), or severe bacterial pneumonia. Influenza, measles, fungal infections, atypical mycobacterial infections, and adenovirus, as well as other viral infections, also can cause and further aggravate bronchiectasis. “These underlying infections may have occurred decades before bronchiectasis symptoms develop,” Dr. Schachter notes.

Bronchiectasis also may occur in people who repeatedly aspirate small amounts of food and saliva or have gastric secretions that enter the lungs due to gastroesophageal reflux disease (GERD). Other bronchiectasis causes include genetic conditions that result in the production of very thick mucus (such as cystic fibrosis) and airway obstructions that prevent proper drainage of mucus. Environmental exposures also may play a role. However, in some people with bronchiectasis, no specific cause is identified (known as idiopathic bronchiectasis).

Bronchiectasis Risk Factors

Certain factors increase your risk for bronchiectasis because they make your airways more vulnerable to infection, inflammation, and scarring and make it harder for you to clear excess mucus. Examples include:

  • Older age, which reduces your immunity to recurrent lung infections and makes it more difficult to cough with sufficient force to clear mucus.
  • Chronic obstructive pulmonary disease (COPD) and asthma, which cause airway damage and increase mucus production.
  • Stroke, Parkinson’s disease, and GERD. “All raise the risk of inhaling foods, liquids, or stomach acid that may enter the lungs and cause an infection,” Dr. Schachter says.
  • Smoking, which exposes the airways to toxic chemicals that promote inflammation, damage the cilia, and contribute to mucus buildup.
  • Cystic fibrosis, an inherited genetic disease that causes mucus to be thick and sticky. It usually presents in childhood but sometimes develops more slowly and presents later in life. “Because this mucus is difficult to expel, it builds up in the lungs, causing inflammation and stimulating bacterial growth,” Dr. Schachter explains. “Mucus plugs also may develop and obstruct the smaller airways.”
  • Primary ciliary dyskinesia, a disorder that affects cilia, resulting in poor mucus clearance and recurrent lung infections.
  • Alpha-1 antitrypsin deficiency, which raises the risk of COPD by significantly reducing levels of a protein that protects the lungs from damage by immune system enzymes.
  • Rheumatoid arthritis, Sjögren’s syndrome, and inflammatory bowel disease (particularly ulcerative colitis), which can sometimes cause chronic inflammation that damages airway walls.
  • Exposure to toxic gases, such as chlorine gas or ammonia.

How Is Bronchiectasis Diagnosed?

Bronchiectasis diagnosis can be tricky because its symptoms, particularly in its earlier stages, resemble those of other lung diseases (keep in mind that it can occur alongside other lung diseases, such as COPD and asthma). To tease out what is causing your symptoms, your doctor will look for specific patterns, such as untreated or recurrent lung infections. “They will use a stethoscope to listen for a phenomenon called rales: crackling sounds produced by air as it moves through mucus in the airways,” Dr. Schachter says. “A sputum sample will be examined for the presence of white blood cells, which are a sign of infection, and may be sent for culture to identify specific bacteria that occur more frequently in people with bronchiectasis.” One such bacterium is Pseudomonas.)

A computed tomography (CT) scan of the chest usually is performed to confirm the diagnosis—if you have bronchiectasis, the CT scan will reveal abnormally wide airways in your lungs. Spirometry (a test of lung function) is not needed to make a diagnosis, but it may be used to assess your airway limitation (particularly when an exacerbation occurs) and to determine disease progression. Additional diagnostic tests may be used to identify specific underlying causes of bronchiectasis.

Bronchiectasis Treatment Options

Bronchiectasis treatment is based in part on its underlying cause. “Treatment will not cure the condition once it is established, but it will make it easier for you to live better with your decreased lung function,” Dr. Schachter observes. “For the best results, see your treatment regimen as ongoing. It isn’t something you can opt in and out of periodically.”

Take steps to limit your exposure to irritants, including air pollution and tobacco smoke. Get the annual flu vaccine and any COVID-19 booster shots your doctor recommends. Also speak with your doctor about getting immunized against pneumonia.

Take antibiotics as directed by your doctor if you develop a respiratory infection. You may require repeat or continuous antibiotic therapy to prevent recurrent lung infections and may require more than one type of antibiotic (sometimes used in rotation to prevent development of germ resistance). In some cases, antibiotics may need to be delivered through a nebulizer (a machine that turns the liquid form of a drug into a fine mist that can be inhaled through a mask or mouthpiece). “Inhaling the drug decreases bacterial growth in the airways,” Dr. Schachter explains.

A new drug called brensocatib (Brinsupri) is the first medication approved by the U.S. Food & Drug Administration to treat airway inflammation in non-cystic fibrosis bronchiectasis occurring after age 12. Mucus-thinning drugs also may be prescribed. If you also have COPD or asthma, medications prescribed for these conditions can be beneficial, as can treatments aimed at treating specific underlying causes like cystic fibrosis, alpha-1 antitrypsin deficiency, and rheumatoid arthritis.

Airway “cleansing” by several methods may be recommended. Daily airway cleansing with concentrated salt solutions delivered by nebulizer sometimes is recommended and helps people cough up mucus. Another approach—bronchoscopic airway clearance therapy and bronchoalveolar lavage (B-ACT)—is performed in the doctor’s office and requires light sedation. It involves insertion of a bronchoscope (a flexible tube with a camera at the tip) through the nose or mouth into the airway and guided to the lungs. The scope is used to vacuum out thick mucus that may be difficult to cough up. Sterile saline solution is then used to flush out bacteria and other toxins. Research has suggested that the technique may extend the length of time between acute exacerbations in people with bronchiectasis and has been shown to reduce sputum production, improve quality of life and lung function, and reduce coughing.

Chest physical therapy is helpful and involves postural drainage with clapping of the chest. Postural drainage involves assuming positions that allow gravity to help mucus flow from the smaller airways into larger airways, from which it may be easier to cough up. “These positions vary depending on which parts of the lungs are worst affected by bronchiectasis, and you may need to use pillows to support your body,” Dr. Schachter says. “You’ll also be shown how to tap your chest with a cupped hand to help break up the mucus.”

Since the strategy can be tiring, your doctor may recommend using an airway clearance device, which includes vibrating devices that are designed to be held against the chest, special breathing devices, and vibrating therapy vests. Small inhalation devices that create vibrations in the airways to loosen secretions are also used. Medicare Part B covers these devices as durable medical equipment if they are prescribed by your physician.

Formal pulmonary rehabilitation programs are very helpful for people with bronchiectasis and include supervised exercise, guidance on breathing exercises and airway clearance, advice on medication management, and nutrition counseling. Most health insurance plans cover pulmonary rehabilitation, though it is advisable to check first. Medicare covers pulmonary rehab only for people with COPD and long COVID, so you will have to be diagnosed with both conditions to be covered for a program. If you have bronchiectasis only, Medicare will cover physical therapy, so your doctor probably will refer you to a therapist who can show you breathing and coughing techniques that help you cough mucus up from your lungs, along with postural drainage techniques.

When airway damage is confined to, or is significantly more severe in, one specific area of the lung, antibiotics are insufficient, and other measures to control bronchiectasis are not successful, the damaged area of the lung may be surgically removed. In certain individuals with severe bronchiectasis, lung transplantation may be an option.

Possible Complications of Bronchiectasis

Many people with bronchiectasis suffer recurrent lung infections and exacerbations. Airway bleeding also may occur, causing you to cough up blood-streaked sputum as a result of the airway damage. “Lung function may decline to a point at which long-term oxygen therapy is necessary,” Dr. Schachter says. “It also is possible for mucus to accumulate and form a plug that causes a section of the lung to collapse. This is most likely to occur in older adults and people with impaired mobility.”

Your cardiovascular health also can be affected by bronchiectasis, as low oxygen levels cause blood vessels in the lungs to narrow. This phenomenon can increase blood pressure in the lung circulation (known as pulmonary hypertension), which means the heart has to work harder to pump blood to the lungs. Over time, right-sided heart failure may develop.

Quality of life also suffers if bronchiectasis is not well controlled. You’ll feel exhausted most or all of the time, may lose weight, and may develop depression.

How to Manage Bronchiectasis Day to Day

Many people successfully manage bronchiectasis for decades. You’ll have the best chance of being one of them if you closely follow your doctor’s advice for managing your airway limitation, take prescribed treatments as directed, and use the recommended approaches for clearing mucus from your lungs. Also follow these strategies to live better with the condition:

  • Drink enough fluids, since staying hydrated can help keep mucus thin enough to cough up more easily. If you have heart failure or kidney disease, clarify with your doctor how much fluid you can safely consume.
  • Engage in airway-clearance exercises each day—don’t be tempted to skip them on days when you feel well.
  • Stay as active as you can, since exercise will help you maintain your lung function as well as preserve your strength and endurance and boost your mood.
  • Avoid smoking and secondhand smoke. If you smoke and are finding it difficult to quit, your doctor can provide advice on smoking cessation counseling and aids. Avoid places where you may encounter secondhand smoke. Also avoid outdoor pollution if at all possible. The Environmental Protection Agency’s AirNow website (www.airnow.gov) provides real-time information on local air quality.
  • Protect yourself against recurrent lung infections by getting annual seasonal vaccines, frequently washing your hands, and avoiding crowded areas if viral outbreaks are affecting your community.
  • Keep a daily record of your symptoms. Knowing what’s “normal” for you might make it easier to identify changes that signal an impending exacerbation.
  • Reach out to support groups when your mood is low. Speaking to others who understand what you are going through can be helpful, and they also can be a good source of useful strategies. The Bronchiectasis & NTM Association has an online community (bronchandntm.org).

When to Call a Doctor

Signs that warrant an immediate call to your doctor include:

  • A sudden worsening of your usual bronchiectasis symptoms, such as more labored breathing, coughing up blood when you have not previously done so (or coughing up a larger amount of blood than before), or producing noticeably thicker sputum, especially if it is a darker color than usual and/or smells unpleasant.
  • A sudden and obvious difference in your exercise tolerance.
  • Signs of a possible respiratory infection, such as a sudden fever, chills, aches and pains, and a sore throat.
  • Chest pain that is more severe or different from the discomfort you experience due to coughing (for example, chest pain that radiates to the arm can signal a heart attack).
  • A rapid heart rate or palpitations (the sense that your heart is missing beats or “skipping” in your chest).
  • A bluish tint to the lips and fingertips, which may indicate hypoxia, a sign of low oxygen levels in the blood.
  • Confusion, disorientation, and extreme drowsiness.
  • Signs of dehydration, such as urinating less often and urine that is darker than normal.
  • Lack of appetite, especially if it is accompanied by weight loss.

Bronchiectasis vs. Bronchitis

People often confuse bronchiectasis for the similar sounding bronchitis, but despite the fact that both are inflammatory conditions, they are different in how they affect the structure of the lungs.

“Bronchiectasis is a chronic disease that permanently scars and widens the airways,” Dr. Schachter says. “Bronchitis causes inflammation in the lining of the airways, but in most cases, the condition resolves after a few weeks and no lasting airway damage occurs.”

He adds that in some cases, bronchitis persists to becomes a chronic condition that results in recurring excess mucus and coughing. “In its chronic form, bronchitis may be a sign of COPD and may contribute to the development of bronchiectasis,” he notes.

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