What is bronchiectasis?

Bronchiectasis is a chronic disease of the lungs. It is caused by repeated damage to the airways of the lungs (bronchi), often as a result of lung infections.

The risk of bronchiectasis increases with age and is twice as common in women as in men. In children, however, boys are more at risk than girls.

Bronchiectasis rates vary widely between populations. Some populations, including indigenous Australians, are more susceptible to it.

Infections

Entry into the body of microorganisms that can reproduce and cause disease.

Lungs

The pair of organs in the chest responsible for breathing.

Causes

Bronchiectasis is the result of a cycle of infection and inflammation. Repeated lung infections cause episodes of inflammation in the lung airways (bronchi). These gradually cause damage and as damage builds up, the airways become wide and riddled with scar tissue.

Many organs and tissues of our body have glands that secrete mucus. This watery substance forms layers that keep the linings of our internal organs moist; it also helps trap and remove harmful foreign substances and microbes.

Mucus is always found inside the airways of the lung, and is cleared away by the movement of cilia - microscopic hairs on the cells that line the airways. When these cells are damaged by inflammation and scarring, mucus is not cleared away fast enough and starts accumulating inside the airways.

This mucus layer provides good breeding grounds for infecting bacteria or fungi. Thus, a new cycle of infection and inflammation starts, which makes the situation worse.

In bronchiectasis, a cycle of infection and inflammation causes mucus to build up inside airways in the lung. 

In about half of bronchiectasis cases, the cause is unknown. Causes and risk factors for bronchiectasis include: [1] [2] [3]

  • Bacterial and fungal infections;
  • A history of childhood infections;
  • Airway obstruction - an inhaled foreign object (often in childhood), or another type of mass blocking an airway in the lungs;
  • Inhaled toxic fumes or smoke;
  • Cystic fibrosis - this causes about a third of all cases of bronchiectasis;
  • Primary ciliary dyskinesia (PCD) - a condition in which the cilia function poorly;
  • Allergic broncho-pulmonary aspergillosis (ABPA) - an allergy to the common fungus Aspergillus fumigatus;
  • A weak immune system;
  • Autoimmune conditions, including Crohn's disease, rheumatoid arthritis, or Sjögren's syndrome, inflammatory bowel disease (IBD), lupus, and;
  • Congenital bronchiectasis - a few newborns are born with bronchiectasis. This is thought to be a result of a problem with fetal development of the lungs.

Allergy

A harmful, hypersensitive immune reaction to usually innocuous environmental substances.

Autoimmune conditions

A medical condition in which the body's immune system abnormally targets substances that are normally found within the body.

Bacteria

Microscopic, single-celled organisms with DNA but no definite nucleus. Bacteria are the cause of many human diseases.

Congenital

Present from birth.

Fungi

An organism from the fungi kingdom, which is a separate group to plants or animals, and includes yeasts, moulds and mushrooms. Fungi feed on organic matter.

Glands

Any organ of the body that secretes substances, such as hormones or enzymes, that are used by other parts of the body.

Immune system

The organs and cells involved in protecting the body against infection.

Infection

Entry into the body of microorganisms that can reproduce and cause disease.

Inflammation

A body’s protective immune response to injury or infection. The accumulation of fluid, cells and proteins at the site of an infection or physical injury, resulting in swelling, heat, redness, pain and loss of function.

Inflammatory bowel disease

A group of conditions characterised by chronic, episodic inflammation of the bowels, including Crohn's disease and ulcerative colitis.

Lungs

The pair of organs in the chest responsible for breathing.

Mucus

A thick, viscous liquid that is secreted for lubrication and to form a protective lining over certain tissues.

Microbes

A microorganism, such as bacteria or fungi, that can cause disease.

1. McDonnell, M.J., Ward, C., Lordan, J.L., et al. (2013) Non-cystic fibrosis bronchiectasis. QJM: An International Journal of Medicine 106:709–715.

2. Boyton, R.J. (2012) Bronchiectasis. Medicine 40:267–272.

3. Barker, A.F. (2002) Bronchiectasis. New England Journal of Medicine 346:1383–1393.

Signs and symptoms

Signs and symptoms of bronchiectasis include: [1] [2]

  • Persistent cough, often with phlegm, lasting for months or years;
  • Coughing up blood;
  • Shortness of breath, wheezing;
  • Fatigue;
  • Chest pain;
  • Pale or bluish skin colour;
  • Clubbing of fingers;
  • Weight loss;
  • Chronic bad breath, and;
  • Feeling unwell.

Fatigue

A state of exhaustion and weakness.

Wheezing

Breathing with a whistling or rattling sound in the chest.

Phlegm

A thick, viscous substance secreted by the lining of the throat and airways, and/or from the lungs.

1. McDonnell, M.J., Ward, C., Lordan, J.L., et al. (2013) Non-cystic fibrosis bronchiectasis. QJM: An International Journal of Medicine 106:709–715.

2. Boyton, R.J. (2012) Bronchiectasis. Medicine 40:267–272.

Methods for diagnosis

Your doctor will diagnose bronchiectasis based on your symptoms and tests, including: [1] [2] [4]

After diagnosing bronchiectasis, your doctor may do further tests to find out its cause. These tests may include:

Blood tests

During a blood test, blood can be drawn using a needle or by a finger prick. Your blood can then be analysed to help diagnose and monitor a wide range of health conditions.

Microorganisms

Any living organism that is too small to be seen clearly with the naked eye, such as bacteria and viruses.

X-ray

A scan that uses ionising radiation beams to create an image of the body’s internal structures.

Sputum culture test

A test carried out on the mucus from the airways of a patient suspected of having a respiratory infection, to determine the presence of disease-causing organisms.

High-resolution computed tomography

A three-dimensional view of the body's organs and structures created from extremely high-definition X-rays.

Lung function tests

Tests used to determine breathing function by measuring lung airflow rates, capacity and volume. Also known as pulmonary function test.

1. McDonnell, M.J., Ward, C., Lordan, J.L., et al. (2013) Non-cystic fibrosis bronchiectasis. QJM: An International Journal of Medicine 106:709–715.

2. Boyton, R.J. (2012) Bronchiectasis. Medicine 40:267–272.

4. Murtagh, J., MD. (2011) John Murtagh’s General Practice (5th Revised edition edition.). North Ryde, N.S.W.: McGraw-Hill Medical Publishing.

Types of treatment

Treatment of bronchiectasis focuses on clearing the excess mucus from the airways and treating the underlying cause of the condition:

Airway clearance

Physical therapy

There are several therapy techniques that can help to physically clear the lungs and airways of mucus. This is done on a regular basis, usually once a day.

The treatment generally involves a carer or health professional thumping the chest area in a variety of ways and postures. Breathing exercises are also used, as well as devices that use vibration and air compression to help dislodge and clear mucus from the airways.

Pulmonary rehabilitation

This is a program of exercises and techniques that aims to reduce symptoms and help a person with lung problems to keep functioning in daily life [1] [5] . Pulmonary rehabilitation exercises can include some or all of the following:

  • Endurance exercise (walking or riding a stationary bicycle);
  • Light weight training or other resistance exercises;
  • Breathing exercises and techniques;
  • Patient education - advice on how to manage your condition, and;
  • Peer support sessions.

Exercise

Exercise of any kind reduces inflammation, encourages air flow and helps loosen the mucus in the respiratory system and control its build-up. Exercise can also develop and maintain the joints and muscles of the chest and back, which help you to breathe.

Medication

Medication to treat respiratory problems in cystic fibrosis can be divided into five categories:

  • Antibiotics that help fend off the infections that are causing bronchiectasis. The specific treatment given depends on the type of infection;
  • Bronchodilators, such as inhaled salbutamol, which widen the airways and help with breathing;
  • Mucus clearance agents, such as saline or mannitol, which help dilute the mucus and help move it out of the airways, and;
  • Anti-inflammatory drugs such as corticosteroids, which can be given to reduce inflammation and swelling within the airways.

Surgery

In serious cases of bronchiectasis, surgery to remove part of the lung may be recommended. Lung transplantation is also an option in extreme cases.

Antibiotics

Chemical substances that kill or suppress the growth of bacteria.

Corticosteroids

A medication that resembles the cortisol hormone produced in the brain. It is used as an anti-inflammatory medication.

Infections

Entry into the body of microorganisms that can reproduce and cause disease.

Inflammation

A body’s protective immune response to injury or infection. The accumulation of fluid, cells and proteins at the site of an infection or physical injury, resulting in swelling, heat, redness, pain and loss of function.

Lungs

The pair of organs in the chest responsible for breathing.

Mucus

A thick, viscous liquid that is secreted for lubrication and to form a protective lining over certain tissues.

Respiratory system

The bodily parts comprising mainly the lungs and the airways. Its primary function is the intake and exchange of oxygen and carbon dioxide gases, respectively.

Respiratory

Relating to respiration, the process of inhaling and exhaling air.

1. McDonnell, M.J., Ward, C., Lordan, J.L., et al. (2013) Non-cystic fibrosis bronchiectasis. QJM: An International Journal of Medicine 106:709–715.

5. Holland, A.E. and Swigris, J.J. (2014) The role of pulmonary rehabilitation and supplemental oxygen therapy in the treatment of patients with idiopathic pulmonary fibrosis. In K. C. Meyer and S. D. Nathan (eds.), Idiopathic Pulmonary Fibrosis. Humana Press.

Potential complications

Complications of bronchiectasis can include:

  • Chronic pneumonia;
  • Collapsed lung (atelectasis) - a lung collapses on itself and is unable to function;
  • Respiratory failure - the lungs are not able to provide enough oxygen to the body, and;
  • Heart failure - the heart is not able to pump enough oxygen-rich blood to the body.

Collapsed lung

When air enters the space between the lung and the wall of the chest (the pleural space), the lung collapses, making breathing difficult. This can occur due to injury or as a result of lung disease.

Lungs

The pair of organs in the chest responsible for breathing.

Respiratory

Relating to respiration, the process of inhaling and exhaling air.

1. McDonnell, M.J., Ward, C., Lordan, J.L., et al. (2013) Non-cystic fibrosis bronchiectasis. QJM: An International Journal of Medicine 106:709–715.

5. Holland, A.E. and Swigris, J.J. (2014) The role of pulmonary rehabilitation and supplemental oxygen therapy in the treatment of patients with idiopathic pulmonary fibrosis. In K. C. Meyer and S. D. Nathan (eds.), Idiopathic Pulmonary Fibrosis. Humana Press.

Prognosis

Bronchiectasis cannot be cured, but it can be managed, allowing people with the condition to live a relatively normal life, especially if the condition is diagnosed and treated early on. However, bronchiectasis has been associated with increased mortality rates. [6]

6. Loebinger, M.R., Wells, A.U., Hansell, D.M., et al. (2009) Mortality in bronchiectasis: a long-term study assessing the factors influencing survival. European Respiratory Journal 34:843–849.

Prevention

Prevention of bronchiectasis focuses on preventing the recurring lung infections that lead to it. Vaccinations, avoiding infection, or treating infections promptly when they occur, are all helpful.

In the past, bronchiectasis was more common, but many of the infections that led to it - such as whooping cough, measles, tuberculosis, and influenza - are much less common now due to routine vaccination programs.

Infections

Entry into the body of microorganisms that can reproduce and cause disease.

Vaccinations

The practice of administering a vaccine, a solution containing a microorganism (that causes a specific disease) in a dead or weakened state, or parts of it, for the purpose of inducing immunity in a person to that microorganism.

6. Loebinger, M.R., Wells, A.U., Hansell, D.M., et al. (2009) Mortality in bronchiectasis: a long-term study assessing the factors influencing survival. European Respiratory Journal 34:843–849.