Bronchiectasis

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 daily symptoms.

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

When to see your GP

You should see your GP if you develop a persistent cough. While this may not be caused by bronchiectasis, it requires further investigation.

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

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 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 many reasons why this may happen. The three most common causes 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 many cases of bronchiectasis, no obvious cause for the condition can be found (known as idiopathic bronchiectasis).

Who is affected

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

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

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 haven’t been effective, the damage to your bronchi is confined to a small area and you’re in good general health.

Possible complications

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

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 have a normal life expectancy. For people with very severe symptoms, however, bronchiectasis can be fatal if the lungs stop working properly.

Symptoms of bronchiectasis

The most common symptom of bronchiectasis is a persistent cough that brings up a large amount of phlegm on a daily basis.

The phlegm can be clear, pale yellow or yellow-greenish in colour. Other people may only occasionally cough up small amounts of phlegm, or none at all.

Other symptoms may include:

• shortness of breath
• wheezing
• coughing up blood or blood-stained phlegm
• chest pain
• joint pain
• clubbing of the fingertips – the tissue beneath the nail thickens and the fingertips become rounded and bulbous

Signs of a lung infection

If you develop a lung infection, your symptoms 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 haven’t already done so
• experience a sharp chest pain that’s 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 requires further investigation.

If you’ve been previously diagnosed with bronchiectasis and you begin to experience symptoms that suggest you have a lung infection, contact your GP. You’ll usually need treatment with antibiotics.

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 38C (100.4F) 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 immediately. This may be your GP, a doctor who specialises in lung conditions (pulmonologist) or a specialist nurse.

 

Causes of bronchiectasis

Bronchiectasis is caused by the airways of the lungs becoming damaged and widened. This can be due to an infection or another condition. Sometimes, the cause is not known.

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 the 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:

• severe pneumonia
• whooping cough 
• tuberculosis (TB) 
• measles

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 because of problems with the genes they inherit from their parents. It’s also possible to acquire an immunodeficiency after 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 caused by Young’s disease and 1 or 2 in every 100 cases are caused by 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.  

Connective tissue diseases

Certain conditions that cause inflammation in other areas of the body are sometimes associated with bronchiectasis, including:

• rheumatoid arthritis
• Sjögren’s syndrome
• Crohn’s disease 
• ulcerative colitis

These conditions are usually thought to be caused by a problem with the immune system, where it mistakenly attacks healthy tissue.

 

Treating bronchiectasis

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

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

There are a number of things you can do to help relieve the symptoms of bronchiectasis and stop the condition getting worse, including:

• stop smoking (if you smoke)
• having the flu vaccine every year
• making sure you have had the pneumococcal vaccine to protect against pneumonia
• exercising regularly
• keeping yourself well hydrated
• eating a balanced diet

Exercises

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

You can be referred to a physiotherpist, 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 involves 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 haven’t first been taught the steps by a suitably trained physiotherapist, as performing the techniques incorrectly could damage your lungs.

If you’re otherwise in good health, you’ll 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 handheld devices that can help to 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 aren’t always available on the NHS, so you may have to pay for one yourself. They usually cost £45 to £60.

Medication

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

Nebulized medications

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

Nebulizers 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 nebulizer, including salt water solutions. These medications help to reduce the thickness of your phlegm so it’s easier to cough it out. Nebulizers can also be used to administer antibiotics, if necessary (see below).

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’ll 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’ll 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’re well enough to be treated at home, you’ll probably be prescribed two to three antibiotic tablets a day for 10-14 days. It’s 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) 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 to 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 won’t be used if more than one lobe is affected, as it’s too dangerous to remove so much lung tissue.

Complications of bronchiectasis

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 amounts 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 think someone is experiencing massive haemoptysis, then phone 999 for an ambulance.

A person with massive haemoptysis needs 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 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.