Bronchiectasis

Bronchiectasis is a chronic lung condition where the airways become permanently widened and damaged, making it difficult to clear mucus and leading to frequent infections that can worsen over time.

Table of contents

• What is bronchiectasis?
• Associated anatomy
• Types of bronchiectasis
• Who is affected
• Symptoms
• Causes
• How bronchiectasis is diagnosed
• Treatment
• Living with bronchiectasis
• Outlook

What is bronchiectasis?

Bronchiectasis is a condition that occurs when the tubes that carry air in and out of your lungs get damaged, causing them to widen and become loose and scarred. These tubes are called airways or bronchi^[1][3].

When your airways are damaged, they slowly lose their ability to clear out mucus, which is a thick substance that removes germs, dust, and other tiny particles from the air we breathe. This mucus buildup creates an environment where harmful bacteria can grow more easily, leading to repeated serious lung infections. The injured airways and infections can become a cycle where each infection causes more damage to your airways^[3].

Bronchiectasis cannot usually be cured, but treatment can help manage symptoms and prevent further damage. Early diagnosis and treatment are important because the sooner your healthcare provider starts treating bronchiectasis and any underlying conditions causing it, the better the chances of preventing further lung damage^[3][4].

• Airways (bronchi)
• Lungs

Types of bronchiectasis

Healthcare providers categorize bronchiectasis in different ways based on what the damage to your airways looks like and where it occurs^[1].

Based on appearance, bronchiectasis can be:

• Cylindrical (or tubular): The most common and least serious form of bronchiectasis
• Varicose: A moderate form
• Cystic: The most severe form

Based on location, bronchiectasis can be:

• Focal: Affecting only one area of the lungs
• Diffuse: Affecting many areas throughout your lungs

Traction bronchiectasis is a specific type that happens when scarring in your lungs pulls your airways out of shape^[1].

Who is affected

About 350,000 to 500,000 people in the United States have bronchiectasis. This includes one person out of every 150 people who are 75 years old and older. The actual number might be higher since you can have bronchiectasis without having any symptoms^[1].

You are at higher risk of bronchiectasis if you are living with an underlying condition that affects your lungs or immune system. Without an underlying condition, your risk increases as you get older^[1].

Research suggests that bronchiectasis disproportionately affects women and older individuals^[5].

Symptoms

The main symptoms of bronchiectasis develop over time and may occur months or years after the event that causes the condition^[1][6].

The most common symptoms include:

• Cough with lots of mucus and pus that does not go away
• Coughing up large amounts of foul-smelling, yellow or green mucus daily
• Repeated chest infections where your symptoms get worse for a few days or weeks
• Shortness of breath that gets worse with exercise
• Wheezing
• Chest pain
• Feeling very tired or run down

Other symptoms that may occur include:

• Coughing up blood
• Bad breath
• Low-grade fever and night sweats
• Noises when you breathe, such as crackling in your lungs or high-pitched squeaks
• Weight loss
• Swollen fingertips with curved nails (called nail clubbing, which is rare)

You might have stretches of time where your symptoms are not as bad and then have periods when they worsen. These periods of worsening symptoms are called exacerbations or flare-ups^[1][4][6].

Causes

Although the cause of bronchiectasis is unknown in many cases, it usually results from an infection or other condition that injures the walls of your airways or keeps the airways from clearing the mucus they make^[3].

Common causes and conditions associated with bronchiectasis include:

Infections

• Severe or repeated lung infections during childhood, such as pneumonia, whooping cough, measles, or tuberculosis
• Bacterial infections from organisms like Haemophilus influenzae, Pseudomonas aeruginosa, Staphylococcus aureus, or Mycobacterium
• Viral infections including respiratory syncytial virus and measles
• Fungal infections
• HIV infection

Genetic and inherited conditions

• Cystic fibrosis (the most common cause in the United States, affecting 50-75% of children with the disease by age 3-5 years)
• Primary ciliary dyskinesia (including Kartagener syndrome)
• Alpha-1 antitrypsin deficiency

Immune system problems

• Immunodeficiency conditions such as common variable immunodeficiency (present in 53% of adults with this condition)
• Hypogammaglobulinemia

Inflammatory and autoimmune diseases

• Rheumatoid arthritis
• Sjögren syndrome
• Inflammatory bowel disease (ulcerative colitis or Crohn’s disease)

Other lung conditions

• Severe chronic obstructive pulmonary disease (COPD) (present in 35-50% of cases)
• Severe asthma (present in 25-40% of cases)
• Allergic bronchopulmonary aspergillosis

Other causes

• Bronchial obstruction from a foreign body, mucus plug, tumor, or enlarged lymph nodes
• Chronic aspiration or gastroesophageal reflux disease
• Inhalational injury
• Breathing in food particles

In roughly 50 percent of cases of non-cystic fibrosis bronchiectasis, no identifiable cause can be found^{[1][3][5][6][8]}.

How bronchiectasis is diagnosed

If you have symptoms of bronchiectasis, your healthcare provider will begin with a thorough evaluation that includes discussing your symptoms, reviewing your personal and family medical history, and performing a physical exam. During the physical exam, your provider will listen to your chest with a stethoscope and may hear small clicking, bubbling, wheezing, rattling, or other sounds, usually in the lower lungs^[1][6].

To confirm a diagnosis, your provider may arrange several tests:

Imaging tests

• Chest X-ray: Technology that uses radiation to produce images of the lungs to check for abnormal lung tissue and thickened airways
• CT scan (computed tomography scan): The main test used to confirm bronchiectasis. It uses specialized X-ray technology to take cross-sectional images that produce detailed 3D pictures showing the location and extent of lung damage

Laboratory tests

• Sputum culture: Testing a sample of your mucus to check for bacteria, fungus, or other sources of infection
• Blood tests: Checking for signs of infection or other conditions that might be causing bronchiectasis, including immune system problems
• Sweat test: Testing for signs of cystic fibrosis

Other tests

• Pulmonary function tests (spirometry): Procedures where you breathe into a machine to measure the amount and rate of air flow in and out of the lungs and how well the lungs move oxygen into the bloodstream
• Bronchoscopy: A minimally invasive procedure using a thin, flexible tube with a tiny light and camera to view the airways and check for blockages or sources of bleeding
• Genetic testing: Tests to check for inherited conditions that may be causing bronchiectasis

If these tests show you could have bronchiectasis, you will be referred to a specialist for more detailed testing^[1][4][6][8].

Treatment

While bronchiectasis cannot usually be cured, the goals of treatment are to manage symptoms, improve quality of life, reduce the frequency of lung infections, remove mucus from the lungs, preserve lung function, and prevent complications^[4][11].

Airway clearance techniques

Keeping your chest clear of mucus is very important and one of the most essential skills you can learn. Everyone with bronchiectasis should see a respiratory physiotherapist to be shown how to use chest clearance techniques. A physiotherapist will teach you breathing techniques for clearing mucus from your lungs and airways and may recommend hand-held devices that use vibrations to help clear your chest. You will usually need to do these exercises every day to help control your cough and reduce the risk of chest infections^[4][13].

Airway clearance methods include:

• Chest physiotherapy involving chest clapping in various positions to move mucus so you can cough it out
• Handheld positive expiratory pressure (PEP) devices that create vibration while breathing
• Percussion devices including mechanical percussors and percussive vests
• Deep coughing and huff coughing techniques

If your mucus is difficult to cough up, your care team may recommend using a machine called a nebulizer to breathe in a salt solution before doing the exercises^[4][13].

Medications

Your doctor may prescribe various medications depending on your symptoms and needs:

• Antibiotics: Used to treat lung infections. You will usually be asked to give your doctor a sample of mucus when you are unwell or if your symptoms change. If you have an infection, you will need to take antibiotics. You may be given antibiotics to keep at home for when you get symptoms of a chest infection. If you get more than three chest infections a year, a specialist may recommend you take antibiotics long term to help prevent them.
• Bronchodilators: Inhaled medications that relax the muscles around your airways to help open them and make breathing easier
• Expectorants and mucus-thinning medicines: Help loosen mucus in your lungs to make it easier to cough up
• Inhaled corticosteroids: May reduce inflammation of the airways and help prevent flare-ups, though they may have side effects
• Macrolides: Antibiotics that reduce inflammation in the airways in addition to killing certain bacteria, sometimes prescribed for long-term use

Pulmonary rehabilitation

Your healthcare provider may refer you to pulmonary rehabilitation, which is a structured series of classes that usually take place over 6-8 weeks. It consists of exercise and education for people with chronic chest conditions and has been shown to have major benefits for people with bronchiectasis. If you have problems with shortness of breath, you may be offered this course led by a physiotherapist^[4].

Oxygen therapy

Oxygen therapy through a mask or tube is reserved for patients who are severely short of oxygen and have severe disease with end-stage complications^[5][11].

Surgery

Surgery is only recommended in extreme cases or to treat the condition causing your bronchiectasis. It may be helpful if medicine does not work and the disease is in a small area, or if you have a lot of bleeding in the lungs. In severe cases, lung transplantation might be needed^[4][6].

Living with bronchiectasis

If you have bronchiectasis, you will need ongoing medical care and should work closely with your healthcare provider to learn how to manage your condition and improve your quality of life. You will have check-ups and tests at least once a year. Your care team will work with you to develop a plan to help you manage your condition^[4][17].

Important daily habits

• Perform airway clearance: Do your chest clearance exercises every day, even when you feel tired. This is the most important skill you can learn to manage bronchiectasis.
• Stay hydrated: Drink plenty of fluids, especially water. This helps keep mucus thin and easier to cough up.
• Exercise regularly: Try to do some exercise every day. Exercise helps clear mucus from your lungs and can give you more energy. Build up gradually and talk to your doctor about the best exercise for you.
• Eat a healthy, balanced diet: A well-balanced diet that includes a variety of vegetables and fruits can help minimize inflammation. Choose whole grains, lean proteins, and foods rich in key vitamins and minerals.
• Get enough rest: Good-quality sleep is important for healing and managing fatigue.

Preventing infections

• Get the annual flu vaccine and the one-off pneumococcal vaccine to help prevent common lung infections
• Get COVID-19 vaccines as recommended
• Wash your hands often, especially before eating, preparing medications, or performing chest clearance
• Avoid people who are sick when possible

Important lifestyle changes

• Quit smoking: This is one of the best things you can do for your lungs. Smokers with bronchiectasis have a higher risk of death from any cause than nonsmokers with the condition. Avoid secondhand smoke as well.
• Avoid toxic fumes and harmful substances: Stay away from gases and other pollutants that can irritate your lungs.

Managing fatigue

It is common to feel very tired when you have bronchiectasis. This tiredness, or fatigue, can be overwhelming. Some tips for conserving energy include using breathing control techniques, pacing yourself by giving yourself more time to do things, organizing your space to make items easier to reach, asking for help when you need it, and practicing relaxation^{[1][4][17][20]}.

When to contact your doctor

Contact your healthcare provider if:

• Your cough is very bad or quickly gets worse
• You are coughing up a lot of mucus every day
• You are coughing up spots or streaks of blood
• You feel short of breath or more short of breath than usual
• You have symptoms of a chest infection, such as a high temperature or chest pain when you breathe or cough
• Other symptoms get worse or do not improve with treatment

Call emergency services immediately if you are coughing up more than just a few spots of blood or have severe difficulty breathing^[4].

Outlook

The outlook for people with bronchiectasis depends on the specific cause of the disease and how much damage has occurred in the lungs. Bronchiectasis can be mild or severe. Some people have it and do not even know it, while others have had it for a long time and repeated infections have severely damaged their lungs^[1][6].

Most people with bronchiectasis live a normal life span by managing their symptoms with a provider’s advice and medication. With treatment, most people live without major disability and the disease progresses slowly. However, frequent exacerbations (flare-ups) have been associated with worse outcomes, including reduced lung function, elevated levels of anxiety, worse health-related quality of life, a higher incidence of hospitalization, and increased mortality. This is why reducing the frequency of lung infections and flare-ups is such an important goal of treatment^[1][6][15].

Early diagnosis and treatment of the underlying cause of bronchiectasis may help prevent further lung damage. The sooner your healthcare provider starts treating bronchiectasis and any underlying conditions, the better the chances of preventing complications^[3][17].