Bronchiectasis

Symptoms

Bronchiectasis is a long-term condition where the airways of the lungs become abnormally widened, leading to a build-up of excess mucus that can make the lungs more vulnerable to infection.

The most common symptoms of bronchiectasis include:

a persistent cough that usually brings up phlegm (sputum)
breathlessness

The severity of symptoms can vary widely. Some people have only a few symptoms that don't appear often, while others have wide-ranging symptoms that occur daily.

The symptoms tend to get worse if you develop an infection in your lungs.

The most common symptom of bronchiectasis is a persistent cough, which affects around 9 out of 10 people with the condition.

Three out of four people with bronchiectasis regularly cough up large amounts of phlegm (sputum), which can be clear, pale yellow or yellow-greenish in colour. Other people may only occasionally cough up small amounts phlegm, or none at all.

Other symptoms include:

shortness of breath
wheezing
coughing up blood or blood-stained phlegm
chest pain

Signs of a lung infection

If you develop a lung infection then your symptoms will usually get worse within a few days. This is known as an infective exacerbation and it can cause:

coughing up even more phlegm, which may be more green than usual or smell unpleasant
worsening shortness of breath

You may also:

feel very tired
cough up blood, if you had not already done so
experience a sharp chest pain that is made worse when breathing (pleurisy)
feel generally unwell

When to seek medical advice

If you haven't previously been diagnosed with bronchiectasis and you develop a persistent cough, visit your GP for advice.

While persistent coughing may not necessarily be the result of bronchiectasis, it will require further investigation.

If you have been previously diagnosed with bronchiectasis and you begin to experience symptoms that suggest you have a lung infection, contact your GP.

This is because it's likely you will need treatment with antibiotics (although some people with bronchiectasis are given a stock of antibiotics as a precaution in case they suddenly develop a lung infection).

When to seek immediate medical advice

Some people with bronchiectasis develop a severe lung infection that may need to be treated in hospital.

Signs and symptoms of serious lung infection include:

a bluish tinge to the skin and lips (cyanosis)
confusion
a high temperature of 38°C (100.4°F) or above
rapid breathing (more than 25 breaths a minute)
severe chest pain that makes it too painful to cough and clear your lungs

If you experience any of the above, phone the healthcare professional in charge of your care for advice immediately. This may be your GP, a doctor who specialises in lung conditions (pulmonologist) or a specialist nurse.

If this isn't possible then phone NHS 111 or your local out-of-hours service.

When to see your GP

You should see your GP if you develop a persistent cough. While this may not necessarily be the result of bronchiectasis, it will require further investigation.

If your GP suspects you may have bronchiectasis, they will refer you to a specialist in treating lung conditions (a respiratory consultant) for further tests.

You should see your GP for advice if you develop a persistent cough so they can look for a possible cause.

Your GP will ask you about your symptoms, such as how often you cough, whether you bring up any phlegm (sputum) and whether you smoke.

They may also listen to your lungs with a stethoscope as you breathe in and out (the lungs of people with bronchiectasis often make a distinctive crackling noise as a person breaths in and out).

You will also probably have a chest X-ray to rule out other, more serious causes of your symptoms, such as lung cancer.

If your GP thinks you may have a lung infection, they may take a sample of your phlegm so it can be checked for bacteria.

Referral to a specialist

If your GP suspects that you could have bronchiectasis, you will be referred to a doctor who specialises in treating lung conditions (a respiratory consultant) for further testing.

The maximum time you should have to wait for referral is 18 weeks, although you may not have to wait as long as this. Read more about waiting times.

Some of the tests a respiratory consultant may carry out to help diagnose bronchiectasis are described below.

HRCT scan

Currently the most effective test available to diagnose bronchiectasis is called a high resolution computerised tomography (HRCT) scan.

A HRCT scan involves taking several X-rays of your chest at slightly different angles, a computer is then used to put all the images together. This produces a very detailed picture of the inside of your body and the airways inside your lungs (the bronchi) should show up very clearly.

In a healthy pair of lungs the bronchi should become narrower the further they spread into your lungs, in the same way that a tree branch will then separate into narrower branches and then twigs.

If the scan shows that a section of airways is actually getting wider, this usually confirms that you have bronchiectasis.

Other tests

Other tests can be used to assess the state of your lungs and to try to determine what the underlying cause of your bronchiectasis may be.

These tests may include:

blood tests – which can be used to check how well your immune system is working and check for infectious agents, such as bacteria, viruses and fungi
a sample of your sweat can be tested to see how much salt is in it – high levels of salt can be caused by cystic fibrosis (if this test is positive then a more detailed genetic test can be carried out; see diagnosing cystic fibrosis for more information)
lung function test – a small, hand-held device (spirometer) that you blow into is used to measure how hard and how quickly you can expel air from your lungs; this can assess how well your lungs are working
bronchoscopy – a flexible tube with a camera at one end is used to look into your lungs; this test is usually only required if it is suspected that you could have inhaled a foreign object

How the lungs are affected

The lungs are full of tiny branching airways, known as bronchi. Oxygen travels through these airways, ends up in tiny sacs called alveoli, and from there is absorbed into the bloodstream.

The inside walls of the bronchi are coated with sticky mucus, which protects against damage from particles moving down into the lungs.

In bronchiectasis, one or more of the bronchi are abnormally widened. This means more mucus than usual gathers there, which makes the bronchi more vulnerable to infection. If an infection does develop, the bronchi may be damaged again so that even more mucus gathers in them and the risk of infection increases further.

Over time, this cycle can cause gradually worsening damage to the lungs.

Why it happens

Bronchiectasis can develop if the tissue and muscles that surround the bronchi are damaged or destroyed.

There are number of reasons why this may happen. The three most common causes in the UK are:

a lung infection during childhood, such as pneumonia or whooping cough, that damages the bronchi
underlying problems with the immune system (the body’s defence against infection) that make the bronchi more vulnerable to damage from an infection
allergic bronchopulmonary aspergillosis (ABPA) – an allergy to a certain type of fungi that can cause the bronchi to become inflamed if spores from the fungi are inhaled

However, in over half of all cases of bronchiectasis no obvious cause for the condition can be found.

Causes

In bronchiectasis, the airways of the lungs become damaged and widened due to inflammation.

Your lungs are continually exposed to germs, so your body has sophisticated defence mechanisms designed to keep the lungs free of infection.

If a foreign substance (such as bacteria or a virus) gets past these defences, your immune system will attempt to stop the spread of any infection by sending white blood cells to the location of infection. These cells release chemicals to fight the infection, which can cause the surrounding tissue to become inflamed.

For most people, this inflammation will pass without causing any further problems. However, bronchiectasis can occur if the inflammation permanently destroys the elastic-like tissue and muscles surrounding the bronchi (airways), causing them to widen.

The abnormal bronchi then become filled with excess mucus, which can trigger persistent coughing and make the lungs more vulnerable to infection. If the lungs do become infected again, this can result in further inflammation and further widening of the bronchi.

As this cycle is repeated, the damage to the lungs gets progressively worse. How quickly bronchiectasis progresses can vary significantly. For some people the condition will get worse quickly, but for many the progression is slow.

Common causes

In around half of all cases of bronchiectasis, no obvious cause can be found.

Some of the more common triggers that have been identified are described below.

Childhood infections

Around a third of cases of bronchiectasis in adults are associated with a severe lung infection in childhood, such as:

However, as there are now vaccinations available for these infections, it is expected that childhood infections will become a less common cause of bronchiectasis in the future.

Immunodeficiency

Around 1 in 12 cases of bronchiectasis occur because a person has a weakened immune system, which makes their lungs more vulnerable to tissue damage. The medical term for having a weakened immune system is immunodeficiency.

Some people are born with an immunodeficiency due to problems with the genes they inherit from their parents. It is also possible to acquire an immunodeficiency due to an infection such as HIV.

Allergic bronchopulmonary aspergillosis (ABPA)

Around 1 in 14 people with bronchiectasis develop the condition as a complication of an allergic condition known as allergic bronchopulmonary aspergillosis (ABPA).

People with ABPA have an allergy to a type of fungi known as aspergillus, which is found in a wide range of different environments across the world.

If a person with ABPA breathes in fungal spores, it can trigger an allergic reaction and persistent inflammation, which in turn can progress to bronchiectasis.

Aspiration

Aspiration is the medical term for stomach contents accidentally passing into your lungs rather than down into your gastrointestinal tract. This is responsible for around 1 in 25 cases of bronchiectasis.

The lungs are very sensitive to the presence of foreign objects, such as small samples of food or even stomach acids, so this can trigger inflammation leading to bronchiectasis.

Cystic fibrosis

Cystic fibrosis is a relatively common genetic disorder where the lungs become clogged up with mucus. The mucus then provides an ideal environment for a bacterial infection to take place, leading to the symptoms of bronchiectasis.

It is estimated that cystic fibrosis is responsible for around 1 in 33 cases of bronchiectasis.

Cilia abnormalities

Cilia are the tiny, hair-like structures that line the airways in the lungs. They are designed to protect the airways and help move away any excess mucus. Bronchiectasis can develop if there is a problem with the cilia that means they are unable to effectively clear mucus from the airways.

Conditions that can cause problems with the cilia include:

Young's disease – a rare condition only affecting males that is thought to be caused by exposure to mercury in childhood
primary ciliary dyskinesia – a rare condition caused by inheriting faulty genes

It's estimated that about 1 in every 33 cases of bronchiectasis are due to Young's disease and 1 or 2 in every 100 cases are due to primary ciliary dyskinesia.

However, as the regulations regarding the use of mercury are now much stricter than they were in the past, it is expected that Young’s syndrome will become a much less common cause of bronchiectasis in the future.

Rheumatoid arthritis

Rheumatoid arthritis is a common condition in which the immune system goes wrong and starts attacking healthy tissue, causing inflammation that in most cases is confined to the joints. However, in a small number of cases the inflammation can spread to the lungs, triggering the symptoms of bronchiectasis.

It is estimated that rheumatoid arthritis is responsible for around 1 in 33 cases of bronchiectasis.

Who is affected

Bronchiectasis is thought to be uncommon. It's estimated that about 1 in every 1,000 adults in the UK have the condition.

It can affect anyone at any age, but symptoms don't usually develop until middle age.

Almost 16,000 people visited hospital in England during 2011-12 with bronchiectasis, of which almost 12,000 were aged 60 or older.

How bronchiectasis is treated

The damage caused to the lungs by bronchiectasis is permanent, but treatment can help relieve your symptoms and stop the damage getting worse.

The main treatments include:

exercises and special devices to help you clear mucus out of your lungs
medication to help improve airflow within the lungs
antibiotics to treat any lung infections that develop

Surgery is usually only considered for bronchiectasis in rare cases where other treatments have been ineffective, the damage to your bronchi is confined to a small area and you are in good general health.

The damage to the lungs associated with bronchiectasis is permanent, but treatment can help prevent the condition getting worse.

In most cases, treatment will involve a combination of medication, exercises you can learn and devices to help clear your airways. Surgery for bronchiectasis is rare.

Exercises

There are a range of exercises, known as airway clearance techniques, which can help remove mucus from your lungs. This can often help improve coughing and breathlessness in people with bronchiectasis.

You can be referred to a physiotherapist who can teach you these techniques.

Active cycle of breathing techniques (ACBT)

The most widely used technique in the UK is called active cycle of breathing techniques (ACBT).

ACBT involve you repeating a cycle made up of a number of different steps. These include a period of normal breathing, followed by deep breaths to loosen the mucus and force it up; then you cough the mucus out. The cycle is then repeated for 20 to 30 minutes.

Don't attempt ACBT if you have not first been taught the steps by a suitably trained physiotherapist, as performing the techniques incorrectly could damage your lungs.

If you are otherwise in good health you will probably only need to perform ACBT once or twice a day. If you develop a lung infection you may need to perform ACBT on a more frequent basis.

Postural drainage

Changing your position can also make it easier to remove mucus from your lungs. This is known as postural drainage.

Each technique can involve several complex steps, but most techniques involve you leaning or lying down while the physiotherapist or a carer uses their hands to vibrate certain sections of your lungs as you go through a series of "huffing" and coughing.

Devices

There are also a number of different handheld devices that can help remove mucus from your lungs.

Although these devices look different, most work in a similar way. Generally, they use a combination of vibrations and air pressure to make it easier to cough out any mucus.

Examples of these devices include the flutter, the RC cornet and the Acapella.

However, these devices are not always available on the NHS so you may have to pay for one yourself. They usually cost between £45 and £60.

Medication

In some cases, medications to make breathing or clearing your lungs easier may be prescribed. These are discussed below.

Nebulised medications

Occasionally, medication inhaled through a device called a nebuliser may be recommended to help make it easier for you to clear your lungs.

Nebulisers are devices consisting of a face mask or mouthpiece, a chamber to convert the medication into a fine mist and a compressor to pump the medication into your lungs.

A number of different medications can be administered using a nebuliser, including salt water solutions. These medications help to reduce the thickness of your phlegm so it's easier to cough it out. Nebulisers can also be used to administer antibiotics if necessary (see below).

However, while the medications used with a nebuliser can be provided on prescription, the nebuliser device itself is not always available on the NHS. In some areas, a local respiratory service may provide the device without charge, but if this is not an option you may have to pay for a device yourself.

Bronchodilators

If you have a particularly severe flare-up of symptoms, you may be prescribed bronchodilator medications on a short-term basis.

Bronchodilators are inhaled medications that help make breathing easier by relaxing the muscles in the lungs. Examples of this type of medication include beta2-adrenergic agonist, anticholinergics and theophylline.

Antibiotics

If you experience a worsening of symptoms because of a bacterial infection (known as an infective exacerbation) then you will need to be treated with antibiotics.

A sample of phlegm will be taken to determine what type of bacteria is causing the infection, although you will be initially treated with an antibiotic known to be effective against a number of different bacteria (a broad spectrum antibiotic) because it can take a few days to get the test results.

Depending on the test results, you may be prescribed a different antibiotic, or in some cases, a combination of antibiotics known to be effective against the specific bacteria causing the infection.

If you are well enough to be treated at home you will probably be prescribed two to three antibiotic tablets a day for 10-14 days. It is important to finish the course even if you feel better as stopping the course prematurely could cause the infection to recur quickly.

If your symptoms are more severe (see symptoms of bronchiectasis for a detailed description) then you may need to be admitted to hospital and treated with antibiotic injections.

Preventative treatment

If you have three or more infective exacerbations in any one year or your symptoms during an infective exacerbation were particularly severe, it may be recommended that you take antibiotics on a long-term basis. This can help prevent further infections and give your lungs the chance to recover.

This could involve taking low-dose antibiotic tablets to minimise the risk of side effects, or using an antibiotic nebuliser (see above for more information about nebulisers).

Using antibiotics in this way does increase the risk that one or more types of bacteria will develop a resistance to the antibiotic. Therefore, you may be asked to give regular phlegm samples to check for any resistance. If bacteria do show signs of developing a resistance then your antibiotic may need to be changed.

Surgery

Surgery is usually only recommended where bronchiectasis is only affecting a single section of your lung, your symptoms aren't responding to other treatment and you don't have an underlying condition that could cause bronchiectasis to recur.

The lungs are made up of sections known as lobes – the left lung has two lobes and the right lung has three lobes. Surgery for focal bronchiectasis would usually involve removing the lobe affected by the bronchiectasis in a type of operation known as a lobectomy.

Surgery will not be used if more than one lobe is affected as it’s too dangerous to remove so much lung tissue.

Possible complications

Complications of bronchiectasis are rare but when they do occur they can be serious. One of the most serious complications is coughing up large amounts of blood due to one of the blood vessels in the lungs splitting. This problem can be life-threatening and may require emergency surgery to treat it.

In some cases, people with bronchiectasis can develop serious complications that require emergency treatment.

Coughing up large amounts of blood

A rare but serious complication of bronchiectasis is coughing up large amount of blood (the medical term for this is massive haemoptysis). This can occur when a section of one of the blood vessels supplying the lungs suddenly splits open.

Symptoms that may indicate massive haemoptysis include:

coughing up more than 100ml of blood in a 24-hour period – 100ml is roughly equivalent to a third of a can of drink
breathing difficulties – caused by blood obstructing your airways
feeling lightheaded, dizzy and having cold clammy skin – caused by rapid blood loss

Massive haemoptysis is a medical emergency. If you suspect that you or someone in your care is experiencing massive haemoptysis then call 999 and ask for an ambulance.

A person with massive haemoptysis will need to be admitted to hospital and a tube may need to be placed into their throat to assist them with their breathing.

A procedure called a bronchial artery embolisation (BAE), carried out by specialist radiology doctors, will then be required to stop the bleeding. During a BAE a special dye is injected into your arteries so that they show up clearly on X-rays.

Then, using X-ray scans as a guide, the source of the bleeding is located and injected with tiny particles, around the size of a grain of sand, that will help clog the vessel up and stop the bleeding.

Outlook

The outlook for people with bronchiectasis is highly variable and often depends on the underlying cause.

Living with bronchiectasis can be stressful and frustrating, but most people with the condition will have a normal life expectancy. In people with very severe symptoms, however, bronchiectasis can be fatal if the lungs stop working properly.

Around 1,000 deaths reported in England and Wales each year are thought to be due to bronchiectasis.

Lungs: Lungs are a pair of organs in the chest that control breathing. They remove carbon dioxide from the blood and replace it with oxygen.
Bacteria: Bacteria are tiny, single-celled organisms that live in the body. Some can cause illness and disease and some others are good for you.