What to know about bronchiectasis

Symptoms

Symptoms are thought to start when sputum builds up in the respiratory system, leading to a cycle of problems.

More sputum means more bacteria in the airways, and this leads to inflammation and airway destruction. Then the cycle begins again with more mucus.

There are three main types of bronchiectasis, classified according to the resulting shape of the bronchi, visible on a CT scan of the lungs.

They are:

• Cylindrical: The most common form, with even, cylinder-shaped bronchi
• Varicose: The least common form. Bronchi are irregular, and the airways may be wide or constricted, leading to a higher production of sputum.
• Cystic: Almost as common as cylindrical, but the bronchi form clusters of cysts. This is the most severe form.

The different types have similar symptoms are similar across the different types, but they differ in terms of severity.

They all feature enlargement of the breathing tubes of the lungs, or bronchi.

Other symptoms include:

• a daily cough that continues for months or years
• daily production of sputum in large amounts
• shortness of breath and wheezing when breathing
• chest pain
• coughing up blood

A person with bronchiectasis who then gets an infection can experience a flare-up, and this can worsen the lung function.

Complications

In time, flares and infections can lead to complications.

Respiratory failure

When too little oxygen transfers from the lungs into the blood, or too little carbon dioxide, a waste gas, is removed from the blood, respiratory failure can occur.

Symptoms include:

• shortness of breath
• rapid breathing
• air hunger, or the constant need for more air
• sleepiness
• bluish skin, fingernails, and lips

Atelectasis

Atelectasis happens when at least one area of the lung fails to inflate properly, leading to shortness of breath, rapid breathing and heart rate, and bluish lips and skin.

Heart failure

At the most advanced stages of bronchiectasis, lung function worsens, putting a strain on the heart. The heart can no longer pump enough blood to meet the body’s needs.

The person may experience:

• trouble breathing
• tiredness
• swelling of the abdomen, neck veins, feet, ankles, and legs

Untreated, it can be fatal.

Causes

Bronchiectasis occurs when a part of the bronchial tree widens irreversibly or dilates.

A wide range of factors can lead to it, including some congenital and autoinflammatory conditions and infections.

Infections that have been linked to bronchiectasis include:

• tuberculosis (TB)
• non-tuberculous mycobacteria
• pneumonia
• childhood infections, such as measles and whooping cough

Immunodeficiency conditions include:

• hypogammaglobulinemia
• leukemia
• HIV and AIDS
• chemotherapy
• other treatments that suppress the immune system

It has also been linked to:

• allergic bronchopulmonary aspergillosis
• obstruction by a tumor or a foreign body
• gastro-esophageal reflux
• inhaling toxic fumes
• auto-inflammatory conditions, such as rheumatoid arthritis, lupus, and ulcerative colitis, or Crohn’s disease
• cystic fibrosis and some other congenital conditions

Between one third and one half of patients appear to have no identifiable cause.

Cystic fibrosis (CF) is a common cause of bronchiectasis in children. This is called CF bronchiectasis. Non-CF bronchiectasis is when the person has bronchiectasis but not CF.

Between 7 and 25 percent of patients with asthma or chronic obstructive pulmonary disease (COPD) also have bronchiectasis, but how these related to bronchiectasis remains unclear.

How does bronchiectasis affect the lungs?

Air passages in the respiratory system make it possible for oxygen to enter the lungs and for carbon dioxide to leave the body.

In healthy lungs, the bronchial tubes narrow smoothly towards the edges of each lung, but in bronchiectasis, they widen and become collapsible and scarred.

The cilia, the hair-like structures that sweep mucus out of the lungs, no longer function ineffective, so the mucus builds up.

This increased mucus provides a place for bacteria to grow. Ongoing infections increase inflammation, and this leads to worsening lung damage.

Is bronchiectasis the same as COPD?

Bronchiectasis, chronic obstructive pulmonary disease (COPD), and cystic fibrosis are classified as obstructive lung diseases.

COPD refers to a collection of lung conditions that make it difficult to breathe, because the airways become inflamed and narrowed. Two conditions that are classified as COPD are persistent bronchitis and emphysema.

Bronchiectasis and COPD are not the same disorder, but studies suggest that between 25 percent and 50 percent of people with COPD also have bronchiectasis.

Diagnosis

A person with an ongoing cough, recurrent lung infections, and sputum in the blood may have bronchiectasis.

Tests may include:

• a chest x-ray
• a CT scan of the lungs
• a lung, or pulmonary, function test (PFT)
• A bronchoscopy, where the doctor uses a lighted tube to look into the lungs, and possibly take a tissue sample

However, laboratory tests do not generally find any specific microorganism in patients that could cause bronchiectasis.

Scientists note that “the bacterial flora appear to change with progression of disease.”

Treatment

Early diagnosis and treatment can help stop the disease from progressing and causing severe complications. Treatment for symptoms can improve the patient’s quality of life.

Many of the treatment options developed have been learned from treating patients with cystic fibrosis.

Treatment aims to:

• deal with underlying conditions or new infections
• remove mucus from the lungs
• prevent complications from developing

There are different forms of treatment.

Chest physical therapy (CPT)

Also known as “chest-clapping” or “percussion,” this is normally carried out by a respiratory therapist.

The patient will either sit down with their head downturned or lie face-down. Gravity helps the mucus to shift.

The therapist repeatedly pounds on the chest and back to loosen mucus and enable coughing. This can be done manually, with the hands, or using a device.

Examples of devices include:

• an electric chest clapper, also known as a mechanical percussor
• an inflatable therapy vest that uses high-frequency airwaves to shift mucus to the upper airway
• a mask that causes vibrations to remove mucus from the walls of the airway

Studies indicate that such techniques may slightly improve the lungs’ ability to get rid of sputum, improve lung function, and enhance quality of life, compared with not using these techniques.

Adding in pulmonary rehabilitation may further improve the ability to exercise and quality of life.

Hydration

Consuming plenty of fluids can help keep mucus thinned out, less sticky and easier to cough up.

Medications

Antibiotics are used to treat infections. They may be given intravenously or by mouth, normally for 14 days. Another possibility is inhaled antibiotics, but these may have adverse effects, and more research is needed into their use.

Expectorants and mucus-thinners can help loosen mucus and support coughing.

Inhaled corticosteroids can treat inflammation of the airways that leads to wheezing or asthma.

A bronchodilator relaxes the muscles around the airways. The medicine is breathed in through an inhaler and nebulizer. Used before CPT, these may increase the benefit of the therapy.

Delivering the bronchodilator directly to the airways enables it to work quickly.

Oxygen therapy

Oxygen therapy, delivered through a mask or nasal prongs, can raise oxygen levels. This can be done at home or in a hospital. It is used in severe cases.

Surgery

Surgery may be suitable if:

• only one part of the airway is affected, so it can be removed
• there is bleeding in the airway that needs to be stopped

Severe cases may require a lung transplant to replace the diseased lungs with a healthy set of lungs.

This is more common if bronchiectasis results from cystic fibrosis.

Outlook and prevention

It is important to seek early treatment for any respiratory condition that could lead to bronchiectasis.

Both adults and children should seek medical help at once if they accidentally inhale a foreign object.

Vaccinations can help prevent measles and whooping cough, childhood diseases that can progress to bronchiectasis.

Avoiding toxic fumes, gasses, and cigarette or other smoke can help preserve respiratory health.

Anyone with a chronic medical condition that increases the risk for bronchiectasis should monitor their lung function and be aware of the early symptoms.