Bronchiectasis

Understanding the airways

Air passes into the lungs via the windpipe (trachea) which divides into a series of branching airways called bronchi.

Air passes from the bronchi into millions of tiny air sacs (alveoli). Oxygen from the air is passed into the bloodstream through the thin walls of the alveoli.

Tiny glands in the lining of the airways make a small amount of mucus. The mucus keeps the airways moist, but also traps any dust and dirt in the inhaled air. There are many tiny 'hairs' (cilia) on the surface of the cells lining the airways. The millions of cilia lining the airways 'sweep' the mucus to the back of the throat to form sputum (phlegm) which is swallowed. Coughing also helps to clear the airways.

What is bronchiectasis?

The airways in bronchiectasis

The extent of bronchiectasis can vary greatly. There may be only one section of one airway that is widened and abnormal. At the other extreme, many airways may be widened. Many affected people fall somewhere between these extremes.

The widened parts of the airways are damaged and inflamed. This causes extra mucus to form which is less easily cleared. These parts of the airways are also more 'floppy' and liable to collapse inwards which may affect air flow through the affected airways. The severity of symptoms depends on how many of the airways are affected, and how badly.

The lung tissue next to a badly inflamed section of airway may also become inflamed and damaged.

What causes bronchiectasis?

The cause is often not clear and no cause can be found in over half of cases. An underlying cause is found in about 4 in 10 cases. Some conditions that affect or damage airways can cause bronchiectasis. Examples include the following:

• Severe lung infections such as tuberculosis (TB), whooping cough, pneumonia or measles can damage the airways at the time of infection. Ongoing bronchiectasis may then develop.
• Some inherited conditions. For example, a condition called primary ciliary dyskinesia affects the cilia so they do not 'beat' correctly to clear the mucus. Cystic fibrosis is another condition that affects the lungs and causes 'bronchiectatic' airways. Some rare immune problems can cause lung infections and damage to airways.
• Inhaled objects, such as peanuts, can become stuck and block an airway. This may lead to local damage to that airway. Acid from the stomach that is regurgitated and inhaled can damage airways. Inhaling poisonous gases may also cause damage.
• Some diseases that cause inflammation in other parts of the body can occasionally cause inflammation and damage in the bronchi and lead to bronchiectasis. For example: ulcerative colitis, Crohn's disease, coeliac disease, rheumatoid arthritis, systemic lupus erythematosus.

What are the symptoms of bronchiectasis?

• Coughing up lots of sputum is the main symptom. The amount of sputum can vary, depending on the severity. It can be very tiring to cough up large amounts of sputum each day.
• Tiredness and poor concentration are common.
• Wheeziness is common.
• Some people become breathless, particularly when exercising or exerting themselves.
• You may cough up some blood from an inflamed airway. This is typically just small amounts of blood now and then. Occasionally, a large amount of blood can be coughed up.
• Some people with bronchiectasis also have chronic (persistent) sinusitis. This may cause an increase in mucus from the nose (catarrh).
• Recurring chest infections are common. This is because an inflamed airway with extra mucus that does not clear easily is ideal for bacteria (germs) to grow and multiply. Sputum turns greeny/yellow when it is infected.

The severity of symptoms varies greatly. Some people have only mild symptoms and become used to an intermittent cough. They may have the occasional chest infection, but a diagnosis of bronchiectasis is never made, or is made years after symptoms begin. At the other extreme, some people have severe symptoms with an almost permanent chest infection. Many people with bronchiectasis fall somewhere in between these extremes.

How is bronchiectasis diagnosed?

If your symptoms suggest bronchiectasis then the diagnosis can be confirmed by a CT scan. A CT scan is like a detailed X-ray test. A CT scan can determine the width of the bronchi. Widened bronchi seen on a CT scan confirm bronchiectasis.

Various other tests may be advised if an underlying cause is suspected.

What are the treatments for bronchiectasis?

Your doctor may advise one or more of the following. The treatment options chosen may depend on the severity of the condition.

Antibiotics

Antibiotics are the 'mainstay' of treatment. If you have mild bronchiectasis, you are likely to need a course of antibiotics every 'now and then' to clear chest infections as and when they occur. A change of the colour of your sputum to green and feeling unwell usually indicates that you have a chest infection.

If you have more severe bronchiectasis, chest infections may return quickly once you stop taking antibiotics. In this situation you may be advised to take antibiotics regularly to prevent infections from developing. One option for this is 'pulsed' antibiotic treatment which is regular short courses of antibiotics with breaks in treatment between the courses. Another option is to take antibiotics every day indefinitely. Recently, taking regular antibiotics by inhaler (nebuliser) rather than antibiotic tablets has become more popular. This is because it can deliver high doses of the antibiotic directly into the airways with relatively small amounts getting into the body. This reduces the problem of possible side-effects.

Physiotherapy and other 'exercise' therapies

The aim of physiotherapy and other exercise therapies is to help you to cough up and clear the mucus, and to improve your overall lung fitness. This may prevent a build-up of infected mucus, which may prevent chest infections. A physiotherapist will advise on the correct way to drain the affected areas. For example, it may mean that once or twice a day you should adopt a 'head down' position and do some controlled breathing exercises to clear the build up of mucus. This is often combined with chest clapping by yourself, or by a relative, carer, or friend. It takes time and effort to do this properly and regularly. More recently, various devices have been designed which help to improve the drainage of mucus.

In addition, if you are able, a daily exercise activity such as running, brisk walking, swimming, dancing, aerobics, etc, may help to clear the mucus too.

Physiotherapy and exercise therapies have been a main treatment for many years. However, research studies to provide evidence in support of these therapies is variable and conflicting. More research is needed to confirm the place of these therapies.

A steroid inhaler

Steroid drugs reduce inflammation. The regular use of a steroid inhaler may reduce inflammation and improve airflow through the airways. Steroid inhalers are the main treatment for asthma (another disease of the airways). Some studies suggest that they may also help with bronchiectasis. For example, a research study published in 2005 described 86 people with bronchiectasis who either took fluticasone 500 μg (a steroid inhaler) twice daily or placebo (a dummy inhaler) for 12 months. The results showed that the amount of sputum coughed up decreased significantly in those treated with fluticasone compared to those who had the placebo inhaler. However, no change was seen in the number of flare-ups of infection, nor in overall lung function. So, this suggests that at least the burden of coughing up large amounts of sputum may be eased with the use of a steroid inhaler.

Other studies have given conflicting results. However, it may well be worth a try with a steroid inhaler. And especially if you have any 'asthma tendency' in addition to bronchiectasis.

Other drugs that have an 'anti-inflammatory' effect are sometimes advised. For example, drugs called leukotriene receptor antagonists may reduce the inflammation in the airways that occurs with bronchiectasis. Further research is needed to look into these other drugs.

Bronchodilator inhalers

Bronchodilator inhalers are commonly used in asthma to 'relax' and open wide (dilate) the airways. There are a number of types, for example, salbutamol inhaler. Studies that have looked into their use in bronchiectasis show conflicting results. They may be of use in some cases. Further research is needed to clarify the role of bronchodilator inhalers for bronchiectasis.

Immunisation

Immunisation against the pneumococcus bacterium and an annual 'flu jab' is advised. This reduces the chance of some types of chest infection developing.

Do not smoke

Smoking makes symptoms worse and smokers are very strongly advised to stop. Also, avoid 'passive smoking' - that is, breathing in the smoke from people smoking nearby.

Treatment of underlying conditions

As mentioned above, about 4 in 10 people with bronchiectasis have an underlying cause. In some of these cases additional treatments may be advised, depending on the cause.

Surgery

An operation may be an option if you have a small local area of lung damage causing symptoms. Cutting out the damaged airway may cure the problem. Surgery may be considered even if you have widespread bronchiectasis. This may be to cut out a particularly bad area of lung that is acting as a reservoir for mucus and infection. Occasionally, a lung transplant may be considered in severe cases.

What is the outlook (prognosis)?

Most people with bronchiectasis (with no underlying cause) have a good outlook. Symptoms in many affected people do not become severe. Treatment, in particular antibiotics when an infection occurs, or regularly when needed, keeps most people reasonably well.

The condition becomes worse in some cases, and breathing problems may develop. In a small number of cases the condition becomes gradually worse over time as more and more of the airways become affected.

A life-threatening bleed from a damaged airway may also occur, but is rare.

The outlook for people where bronchiectasis is part of another condition depends on the underlying cause.