Bronchiectasis Overview

What Is Bronchiectasis?

Bronchiectasis (pronounced bron-kee-ek'-tas-is) is a condition of the airways in the lungs. To understand this disease, it’s important to know how healthy airways work. These airways (bronchial tubes) are tube-like structures that branch from the trachea into the right and left lungs. Air moves in and out of the airways. As the airways branch, like branches on a tree, they get smaller and smaller. The airways are lined with cells that produce mucus. Cilia are microscopic hair like structures along the surface of the airways that beat together to help clear the mucus and bacteria from the lungs.

When a person has bronchiectasis, the airways are permanently and abnormally widened (dilated). The cells lining the airways become inflamed (swollen). These damaged airways can no longer effectively clear mucous and bacteria from the lung. This can lead to exacerbations of cough, sputum production, and shortness of breath.

Bronchiectasis is caused by one or more infectious or inflammatory conditions in the lungs. People with bronchiectasis are more likely to get lung infections. Each lung infection can make the bronchiectasis worse. Therefore, early diagnosis and treatment of bronchiectasis is very important.

Causes

There are many causes of bronchiectasis. Some are acquired, and others a person may be born with (congenital). The following is a list of the most important and/or common ones.

Infections

Infections can damage the airways and cause bronchiectasis. They also may be a consequence of the disorder, which can lead to worsening bronchiectasis. Examples include:

• Viral infections (measles, adenovirus, influenza)
• Bacterial infections (Pseudomonas aeruginosa, Staphylococcus aureus, Klebsiella)
• Mycobacterial infections (tuberculosis, Mycobacterium avium, Mycobacterium abscessus)
• Fungal infections (histoplasmosis)

Immune Diseases

Persons with immune deficiencies, such as antibody deficiencies, are more likely to have repeated lung infections which can damage the airways and cause bronchiectasis.

Autoimmune Diseases

Rheumatoid arthritis, Lupus, Sjogren's syndrome and Granulomatosis with polyangiits (formerly knowns as Wegener's granulomatosis) are examples of rheumatologic, autoimmune or connective tissue diseases that can cause bronchiectasis.

Genetic Diseases

Cystic fibrosis: Cystic fibrosis causes impaired mucus that makes clearance of secretions with bacteria difficult, and this “sticky mucus” leads to recurrent lung infection and bronchiectasis. Classic cystic fibrosis is obvious at birth, but there are forms (certain less common gene mutations) of cystic fibrosis that may not be recognized until adulthood.

Primary ciliary dyskinesia: Primary ciliary dyskinesia impairs the ability of small hairs called cilia to clear mucus and bacteria from the airways. Recurrent lung infections can occur and cause bronchiectasis.

Alpha-1: Alpha-1 Antitrypsin Deficiency  is a protein that moderates inflammation that occurs during an infection. People who are deficient in alpha-1 antitrypsin or who have an abnormal protein may be more likely to have recurrent lung infections that cause bronchiectasis.

Aspiration

Chronic pulmonary aspiration occurs when a person inhales oral or stomach material into his/her lungs. If severe or recurrent, aspiration can lead to inflammation of the airways and cause bronchiectasis. The aspiration can occur from:

• Impaired ability to swallow (oropharyngeal dysphagia) that may cause saliva or food to enter the lung.
• Gastroesophageal reflux disease (GERD) which occurs when the valve of smooth muscle between the esophagus and the stomach does not function properly, and allows stomach contents (acid and non-acid) to flow back up into the esophagus. The stomach contents may enter your lungs and irritate the airways. 

Obstruction of the Airways

Obstructed airways trap mucous and infection behind the obstruction that can damage the airways and cause bronchiectasis. Obstruction of the airways can be caused from a growth such as a tumor or can be from aspiration of a substance such as a pill.

Inflammation of the Airways

Chronic obstructive pulmonary disease (COPD) and allergic bronchopulmonary aspirgillosis are diseases that can cause airway damage with chronic ongoing inflammatory injury.

Signs and Symptoms

Bronchiectasis symptoms may include a cough. The cough may produce mucus. With infections, the mucus may be discolored and foul-smelling and may contain blood (hemoptysis). The cough also may be nonproductive of mucus. The mucus may be in the airways of the lungs, but it may be hard to cough up the mucus.

Other common symptoms of bronchiectasis may include: 

• shortness of breath
• wheezing
• fatigue 

Some people with bronchiectasis also have chronic sinusitis. This requires further evaluation, since bronchiectasis and sinusitis may be due to the same underlying disease. Your doctor will evaluate all your symptoms as part of managing your bronchiectasis.

If left untreated, symptoms of bronchiectasis may progress causing further damage to the airways in the lungs. Further symptoms may include increasing shortness of breath, worsening quality of life and even heart failure.

Risk Factors

Your risk for bronchiectasis may be affected by the following factors: 

• Age: Bronchiectasis is more likely to be diagnosed in those over the age of 65.
• Gender: Bronchiectasis is more commonly diagnosed in women as compared to men. 
• Environment: Toxins and airborne pollutants can cause inflammation, which can lead to bronchiectasis. 

Diagnosis 

The first step in diagnosing bronchiectasis is a thorough evaluation. Your doctor may have you do a number of tests to evaluate your breathing.

A multiple step process usually leads to the diagnosis of bronchiectasis. Many factors are considered. The evaluation for bronchiectasis often includes examining a complete medical history while conducting a complete physical examination. 

Breathing and Exercise Tests

Pulmonary function tests: Pulmonary function testing measures how well you are breathing. There are different types of breathing tests that can be done during pulmonary function testing. They include spirometry, lung volumes and diffusing capacity. Spirometry can show how much air you can breathe in and out. It also shows how fast you can breathe in and out. Lung volumes can provide further information about how your lungs are functioning. Diffusing capacity can show how well your lungs move oxygen from the lungs to the blood. The results of pulmonary function testing can help your doctor find the best treatment plan for you.

Imaging Tests

CT scan of the chest: A CT or CAT scan is a shortened name for computerized tomography. During a CT scan of the chest, detailed pictures are taken of cross sections or slices of the thoracic structures in your body. Thoracic structures include your lungs, heart and the bones around these areas. Sometimes intravenous contrast is administered to better see the blood vessels in the lung.

Sputum cultures: An analysis of several sputum cultures can help your doctor look for infections and must be done at specialized labs. Sputum is the sticky fluid produced in the lungs that is sometimes coughed up. One positive test does not always mean disease is present. 

You and your doctor may choose to have you seen by a specialist, such as a pulmonologist (lung specialist) to confirm a diagnosis and treat you bronchiectasis once it is diagnosed.

Generally the prognosis of bronchiectasis is very good. The earlier it is diagnosed, the earlier treatment and management can be initiated preventing it from worsening. Be proactive with your doctor.

Treatment

The management of bronchiectasis is long-term and is directed at:

• Improving the clearance of sputum, also called airway clearance or bronchopulmonary hygiene
• Treatment of infections
• Reducing inflammation in the airways with certain treatments
• Treatment of associated conditions (such as GERD or sinusitis)
• Improving muscle strength and endurance through pulmonary rehabilitation 
• Identifying the need for surgical resection of affected segments or lobes of the lung

Your doctor will evaluate your history and recommend the best management plan for you.

Medications

Inhaled medications: Inhaled long-acting bronchodilators may also be used. Long-acting bronchodilators can be either LABAs (long-acting beta2 agonists) or LAMAs (long-acting muscarinic antogonists).  LABA and LAMA are types of bronchodilators.

LABAs include:

• Arcapta® (indacaterol)
• Brovana® (arformoterol)
• Perforomist® (formoterol)
• Serevent® (salmeterol)
• Stiverdi® (olodaterol)

LAMAs include:

• Incruse® (umeclidinium)
• Seebri® (glycopyrronium)
• Spiriva® (tiotropium)
• Tudorza® (aclidinium)

LAMA & LABA combinations include:

• Anoro® (umedcldinium and vilanterol)
• Stiolto® (olodaterol and tiotropium)
• Utibron® (indacaterol and glycopyrolate)

Inhaled steroids: Inhaled steroids reduce and prevent swelling inside the airways. They are not a standard in bronchiectasis management, but are sometime used in certain patients depending on other conditions they may have, such as asthma. Common inhaled steroids include:

• Alvesco® (ciclesonide)
• ARNUITY (fluticasone)
• Asmanex® (mometasone)
• Flovent® (fluticasone)
• Pulmicort® (budesonide)
• QVAR® (becolmethasone)

Inhaled steroid and long-acting bronchodilator combinations: The use of inhaled steroids alone or in combination with other medications are not a standard in bronchiectasis management, but are sometime used in certain patients depending on their other conditions, such as asthma and/or COPD. Common combinations of inhaled steroid and long-acting bronchodilator include:

• Advair® (fluticasone and salmeterol)
• Breo® (fluticasone and vilanterol)
• Dulera® (mometasone and formoterol)
• Symbicort® (budesonide and formorterol)

Inhaled hypertonic saline may be used to loosen airway mucus for easier clearance.  Common strengths of hypertonic saline are 3% and 7% and are generally used once or twice daily and are administered with a nebulizer.

Bronchopulmonary Hygiene Therapy: Improved clearance of mucus is the cornerstone of the management of bronchiectasis and includes several components. They include:

• Inhaled medication (bronchodilator with sodium chloride saline) 
• Airway clearance measures (oscillating positive expiratory pressure device, high-frequency chest wall oscillation vest)
• Breathing techniques such as active cycled breathing or autogenic drainage

Your doctor may recommend one or more of them depending on your individual needs.

An inhaled bronchodilator medication opens the airways by relaxing the smooth muscles around the airways. This type of medication is available in a number of inhaled forms. 

Commonly used inhaled short-acting bronchodilators include:

• ProAir RespiClick or HFA®, Proventil® HFA, Ventolin® HFA (albuterol)
• Xopenex® (levalbuterol) 

Lifestyle Management

Making adjustments to your lifestyle can improve your symptoms.

• Exercise regularly as directed by your health care provider. This helps you breathe easier by improving your muscle strength and tone and helps improve clearing the mucus from the airways.
• Eat a well-balanced healthy diet and drink plenty of fluids. If you have GERD you may need to limit fluids, especially 2-3 hours before bed.
• Give up smoking and avoid exposure to passive smoke. Ask your health care provider for techniques to help you give up smoking.
• Get a flu shot every year in the fall. Get the pneumococcal vaccines as recommended by your health care provider.

Living with bronchiectasis is a unique and special challenge that you and your family must deal with on a daily basis. Management is a long-term commitment. But the more you know about bronchiectasis, the better suited you are in managing the various aspects of your disease. As you take control, your quality of life will improve.

Be sure to talk with your healthcare provider if you have questions or concerns about your plan. Write down any questions you have and ask your healthcare provider at your next appointment.

Clinical Trials

Clinical trials help determine new treatment options for diseases and conditions. Patients with bronchiectasis have access to clinical trials and should speak with their physician to determine what trials might work best for them.

See a Specialist

It’s important to be evaluated by a pulmonologist if you or a loved one: 

• Has symptoms of bronchiectasis 
• Has alpha-1 antitrypsin deficiency or cystic fibrosis
• Would like to improve your current treatment of bronchiectasis

At National Jewish Health in Denver, Colorado, w Bronchiectasis Treatment Program treats hundreds of patients each year. Learn more about our program or use the button below to make an appointment.