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Aspergillosis

 

Aspergillosis is a term used for a range of infections caused by a fungal mould called aspergillus.

Aspergillosis usually affects the lungs, but can spread to almost anywhere in the body.

The infection can range from being mild to life-threatening. Symptoms range from mild wheezing to coughing up blood.

Aspergillosis usually only affects people with pre-existing health conditions, as most healthy people have natural immunity to it.

Types of aspergillosis

There are four main types of aspergillosis:

  • allergic bronchopulmonary aspergillosis,
  • aspergilloma,
  • chronic necrotizing aspergillosis, and
  • invasive pulmonary aspergillosis.

The four types are described below.

Allergic bronchopulmonary aspergillosis (ABPA)

Allergic bronchopulmonary aspergillosis (ABPA) is caused by an allergic reaction to spores of fungus mould. It is the mildest form of aspergillosis.

ABPA usually affects people with asthma or cystic fibrosis (an inherited condition where the lungs get plugged up with mucus).

Symptoms include coughing, wheezing and a worsening of asthma symptoms.

Aspergilloma

In aspergilloma, aspergillus fungi enter the lungs and group together to form a dense knot of fungi, called a fungal ball.

Aspergilloma usually only affects people who have a pre-existing lung condition, such as TB, which means that they have cavities (spaces) or damage in their lungs.

Coughing up blood is one of the most common symptoms of aspergilloma. The medical term for coughing up blood is haemoptysis.

Chronic necrotizing aspergillosis (CNA)

Chronic necrotizing aspergillosis (CNA) is a slow spreading, chronic (long-lasting) infection of the lungs by the aspergillus fungus.

CNA usually only affects people with a pre-existing lung condition, or people who have a weakened immune system, as a result of conditions, such as diabetes, liver disease or chronic alcohol abuse (an excessive amount of alcohol can weaken the immune system).

Invasive pulmonary aspergillosis (IPA)

Invasive pulmonary aspergillosis (IPA) is a common infection in people with severely weakened immune systems due to illness or taking immunosuppressants. IPA is the most serious form of aspergillosis.

IPA is a fungal infection in the lungs that then spreads rapidly through the body. The infection can spread to the blood, brain, heart and kidneys.

IPA usually only affects people with a severely weakened immune system, such as:

  • people with end-stage HIV,
  • people who are undergoing high-dose chemotherapy, and
  • people taking medication to suppress their immune system (immunosuppressants) because they have had an organ or bone marrow transplant.

IPA is a medical emergency that requires hospitalisation and prompt treatment with injections of anti-fungal medication.

How common is aspergillosis?

Allergic bronchopulmonary aspergillosis (ABPA)

ABPA is estimated to affect 0.25-0.8% of people with mild to moderate asthma, and 7% of people with cystic fibrosis.

In cases of more severe asthma that can only be controlled with steroid medication, it is estimated that 7-10% of people will have ABPA.

Aspergilloma

Aspergilloma is a common condition in people who have cystic fibrosis or cavities in their lungs.  

Chronic necrotizing aspergillosis (CNA)

Chronic necrotizing aspergillosis (CNA) is thought to be a rare condition, but there is little available data to estimate exactly how many people develop CNA. If the symptoms of CNA are mild, it can go undiagnosed during life.

Invasive pulmonary aspergillosis (IPA)

IPA is estimated to occur in 5-13% of people who have had a bone marrow transplant, 5-25% of people who with a heart or lung transplant and 10-20% of people who undergo high-dose radiotherapy for leukaemia (cancer of the blood cells).

Outlook

The outlook for ABPA is good as most people take steroid medications to prevent the allergic response. However, if ABPA reoccurs, a long-term course of steroids may be required.

The outlook for aspergilloma and CNA is fair. Both can be treated with anti-fungal medications. In some cases, surgery may be required to remove the infection from the lungs. This carries an associated risk of complications, particularly in people whose lungs are already damaged.

There is also a risk that the symptom of haemoptysis (coughing up blood) may become life-threatening, and require emergency surgery to stop the source of the bleeding.

Even though IPA can be treated with anti-fungal medication, the outlook is poor. The infection can spread very quickly, and someone who develops IPA is usually already very ill. An estimated 50% of people do not respond to treatment and die from the condition.

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.

Immune system

The immune system is the body's defence system, which helps protect it from disease, bacteria and viruses.

Allergic

An allergen is a substance that reacts with the body's immune system and causes an allergic reaction.

Wheezing

Wheezing is the whistling sound made during breathing when the airways are blocked or compressed.

Abscess

An abscess is a lump containing pus, which is made by the body during infection.

Chemotherapy

Chemotherapy is a treatment of an illness or disease with a chemical substance, e.g. in the treatment of cancer.

Kidneys

Kidneys are a pair of bean-shaped organs located at the back of the abdomen, which remove waste and extra fluid from the blood and pass them out of the body as urine.

Heart

The heart is a muscular organ that pumps blood around the body.

Brain

The brain controls thought, memory and emotion. It sends messages to the body controlling movement, speech and senses.

Useful Links

Allergic bronchopulmonary aspergillosis (ABPA)

The symptoms of allergic bronchopulmonary aspergillosis (ABPA) include:

  • a high temperature (fever) of 38°C (100°F) or above,
  • shortness of breath,
  • wheezing,
  • fatigue,
  • a cough that brings up mucus,
  • haemoptysis (coughing up blood), and
  • a general sense of feeling unwell.

If you have asthma, your usual symptoms may become more difficult to control with medication.

Aspergilloma

Haemoptysis (coughing up blood) is the most common symptom of aspergilloma. Other symptoms are:

  • a high temperature (fever) of 38°C (100°F) or above, and
  • a cough.

Chronic necrotizing aspergillosis (CNA)

Symptoms of chronic necrotizing aspergillosis (CNA) include:

  • a persistent cough that brings up mucus,
  • haemoptysis (coughing up blood),
  • a high temperature (fever) of 38°C (100°F) or above,
  • unexplained weight loss,
  • night sweats, and
  • a general sense of feeling unwell.

Invasive pulmonary aspergillosis (IPA)

The symptoms of invasive pulmonary aspergillosis (IPA) can vary depending on whereabouts in the body the infection spreads. Symptoms include:

  • a high temperature (fever) of 38°C (100°F) or above,
  • a cough that brings up mucus,
  • haemoptysis (coughing up blood),
  • wheezing,
  • chest pain,
  • shallow, rapid breathing,
  • headache, and
  • fatigue.

When to seek medical advice

Contact your GP and/or your transplant team immediately if you develop symptoms that suggest you may have an infection, such as a fever or persistent coughing, and:

  • you are taking immunosuppressants as a result of having an organ or bone marrow transplant, or
  • you have a condition that is known to weaken the immune system, such as cancer.

Always contact your GP if you have:

  • haemoptysis (coughing up blood),
  • unexplained weight loss, and/or
  • symptoms of persistent coughing and fever.

Glossary

Pain
Pain is an unpleasant physical or emotional feeling that your body produces as a warning sign that it has been damaged.
Congestion
Congestion is an excess of fluid in part of the body, often causing a blockage.
Fever
A high temperature, also known as a fever, is when someone's body temperature goes above the normal 37°C (98.6°F).
Blood
Blood supplies oxygen to the body and removes carbon dioxide. It is pumped around the body by the heart.
Wheezing
Wheezing is the whistling sound made during breathing when the airways are blocked or compressed.

The aspergillus mould that causes aspergillosis is found in all countries and many different environments. It can therefore be difficult to avoid.

Aspergillus mould can be found in:

  • decaying leaves,
  • compost,
  • plants,
  • trees, and
  • crops.

The mould can also grow inside buildings and is found in:

  • air conditioning systems,
  • heating systems,
  • insulation material,
  • carpets,
  • dust,
  • household plants, and
  • ground pepper and spices.

There have been cases of aspergillus mould growing inside computers.

The aspergillosis infection is caught by breathing in small spores of aspergillus mould. Most people's immune systems will quickly isolate and destroy the mould before it has a chance to spread into their lungs.

But if a person has a weakened immune system or damaged lungs, the infection is more likely to stay.

Allergic bronchopulmonary aspergillosis (ABPA)

If allergic bronchopulmonary aspergillosis (ABPA) is suspected, your GP may perform (or refer you for) a skin allergy test.

Your skin will be pricked with a needle that contains a small amount of the aspergillus mould. If you are allergic, the area of skin where the needle was placed will come up in a hard red bump.

Blood tests are also used to check for antibodies that are created by your immune system if you are allergic to the aspergillus mould.

You may also have a computerised tomography (CT) scan to check whether there is a build-up of mucus in your lungs (bronchiectasis), which is common in ABPA.

Aspergilloma

If aspergilloma is suspected, it is likely that you will be referred for chest X-rays and a CT scan. These are both used to check for the presence of a fungal ball, which can show up as a dark mass.

A sample of your mucus may also be taken to check for antibodies and fungal spores.

Chronic necrotizing aspergillosis (CNA)

Chronic necrotizing aspergillosis (CNA) is usually diagnosed by asking you about your symptoms and using X-rays and CT scans to look at your lungs. Samples of your blood and mucus may also be checked for antibodies and fungal spores.

In some cases of CNA, you may need to have a biopsy to confirm the diagnosis. This involves taking a small sample of tissue from your lungs and checking it for fungal spores using a microscope.

A biopsy is usually carried out using an instrument called a bronchoscope, which is a thin tube that is passed down your throat and into your lungs. The bronchoscope is used to remove a sample of lung tissue. The procedure is performed under a local anaesthetic (the area is numbed), so it is not painful and only takes around five minutes to complete.

Invasive pulmonary aspergillosis (IPA)

Invasive pulmonary aspergillosis (IPA) is diagnosed in the same way as CNA, using a combination of CT scans, blood and mucus tests and biopsy.

Glossary

Tissue
Body tissue is made up of groups of cells that perform a specific job, such as protecting the body against infection, producing movement or storing fat.  
Antibodies
Antibodies and immunoglobulins are proteins in the blood. They are produced by the immune system to fight against bacteria, viruses and disease.
Biopsy
A biopsy is a test that involves taking a small sample of tissue from the body so it can be examined.
Lesion
A lesion is an abnormal change in an organ or body tissue because of injury or disease.
Blood
Blood supplies oxygen to the body and removes carbon dioxide. It is pumped around the body by the heart.
X-ray
An X-ray is a painless way of producing pictures of inside the body using radiation.
Lung
Lungs are a pair of organs in the chest that control breathing. They remove carbon dioxide from the blood and replace it with oxygen.

Allergic bronchopulmonary aspergillosis (ABPA)

Allergic bronchopulmonary aspergillosis (ABPA) is treated using corticosteroid tablets (oral corticosteroids).

Corticosteroids suppress the immune system. This is to help prevent the allergic reaction that ABPA can cause.

Most people require a three to six-month course of oral corticosteroids. The dose is gradually reduced over time before treatment is stopped.

The side effects of oral corticosteroids include:

  • osteoporosis (thinning of the bones),
  • high blood pressure (hypertension),
  • diabetes,
  • weight gain,
  • cataracts and glaucoma (eye disorders),
  • thinning of the skin,
  • bruising easily, and
  • muscle weakness.

The following advice may help minimise these side effects:

  • Eat a healthy, balanced diet that includes plenty of calcium. Foods that are high in calcium include dairy products, spinach, salmon, beans and rhubarb.
  • Maintain a healthy body weight.
  • Do not smoke.
  • Do not drink more than the recommended daily limit of alcohol (three to four units a day for men; two to three units a day for women). A unit of alcohol is equal to about half a pint of normal strength lager, a glass of wine or a single shot of spirit.
  • Take regular exercise.

You will also need regular appointments to check for high blood pressure (hypertension), diabetes and osteoporosis when taking corticosteroids.

Aspergilloma

To treat aspergilloma, you may be given injections of an anti-fungal medication called amphotericin B. This can stop haemoptysis (coughing up blood).

An injection is usually given directly into the lungs. The doctor giving the injection will use a CT scanner to guide the needle to the site of the fungal ball. Many people experience side effects one to two hours after having an injection. These include:

  • a high temperature (fever) of 38°C (100°F) or above,
  • shaking,
  • chills,
  • dizziness,
  • loss of appetite,
  • nausea,
  • vomiting,
  • headache, and/or
  • rapid breathing.

These side effects are usually more severe when taking the initial doses, and get better as your body gets used to the medication.

Once haemoptysis (coughing up blood) is controlled, you may be given an additional anti-fungal medication called itraconazole. This is usually given in tablet form.

The length of time that you will have to take itraconazole depends on how well your symptoms react to the medication. Side effects of itraconazole are rare, but may include:

  • headache,
  • nausea,
  • vomiting,
  • skin rash, and
  • hair loss.

Chronic necrotizing aspergillosis (CNA) and invasive pulmonary aspergillosis (IPA)

Due to the similarities between the causes of chronic necrotizing aspergillosis (CNA) and invasive pulmonary aspergillosis (IPA), both of these conditions are treated in the same way.

If you are diagnosed with CNA or IPA, you may be admitted to hospital so that your health can be carefully monitored. You will also receive assistance with your breathing, if required.

CNA and IPA are usually treated with injections of a powerful anti-fungal medication called voriconazole.

Common side effects of voriconazole include:

  • visual disturbances (changes in vision),
  • a high temperature (fever) of 38°C (100°F) or above,
  • a rash,
  • nausea,
  • vomiting,
  • diarrhoea,
  • headache, and
  • stomach pains.

Rarer side effects of voriconazole include:

  • flu-like symptoms such as joint and muscle pain,
  • changes in mental state, such as depression or anxiety,
  • dizziness,
  • hallucinations (seeing or hearing things that are not real),
  • breathing difficulties,
  • jaundice (yellowing of the skin and the whites of the eyes), and
  • swelling of the lips or face.

If you also have a weakened immune system, you may be given additional treatment to help strengthen it. This includes a type of medication known as colony-stimulating factors (CSFs). CSFs help encourage your bone marrow to produce more white blood cells, which fight infection.

Another medication that can be used to boost the immune system is called interferon gamma, which is a genetically engineered version of a protein that the immune system uses to fight off infection.

The most common side effects of interferon gamma and CSFs are flu-like symptoms, such as:

  • a high temperature (fever) of 38°C (100°F) or above,
  • chills,
  • joint and muscle pain,
  • headache, and
  • nausea.

Surgery

Surgery may be required for cases of CNA, IPA and aspergilloma that fail to respond to anti-fungal treatment, or if the symptoms of haemoptysis (coughing up blood) are thought to be life threatening. Extensive haemoptysis can cause the lung tissue to become filled with blood, leading to asphyxiation (suffocation).

Surgical resection

With severe CNA, IPA and aspergilloma, one of the treatment options is the surgical resection of the lung, to remove the infected piece of lung tissue.

Depending on the extent of the infection, the type of surgical resection used could be:

  • a wedge resection, where a small piece of lung is removed,
  • a lobectomy, where the top or bottom half of the lung (known as a lobe) is removed, or
  • a pneumonectomy, where the entire lung is removed.

Many people who have surgical resection are worried that they will not be able to breathe properly afterwards, but it is possible to breathe with just one lung. 

Bronchial artery embolisation

Bronchial artery embolisation (BAE) is a common treatment for life-threatening haemoptysis (coughing up blood). Haemoptysis is caused when one of the major blood vessels in the lungs, the bronchial arteries, are weakened by infection. The weakened artery ruptures (splits), releasing blood. The aim of a BAE is to block the flow of blood out of the artery.

During a BAE, a small, flexible tube (a catheter) is guided into your lungs. A CT scanner is usually used to guide the catheter to the exact site of the rupture.

A gelatine (jelly-like) solution is passed down the catheter. This is to block the site of the rupture and prevent further bleeding.

 

Glossary

Inhaler
Inhaler is a device for delivering a medicine as a vapour or powder into the lungs.
Physiotherapy
Physiotherapy is a treatment that uses physical movements, massage and exercise to relieve illness or injury.
Vein
Veins are blood vessels that carry blood from the rest of the body back to the heart.
Corticosteroids
Corticosteroid is a naturally occurring hormone produced by the adrenal gland, or a synthetic hormone having similar properties. It is used to reduce inflammation, so reducing swelling and pain.
Immune
The immune system is the body's defence system, which helps protect it from disease, bacteria and viruses.
Bronchodilators
Bronchodilator medicines are used to widen the airways of the lungs to help with breathing difficulties. For example, salbutamol.
Allergic
An allergen is a substance that reacts with the body's immune system and causes an allergic reaction.
Intravenously
Intravenous (IV) means the injection of blood, drugs or fluids into the bloodstream through a vein.
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.

Avoid exposure to the aspergillus fungus

The main way to prevent aspergillosis is to avoid exposure to the aspergillus fungus.

If you have asthma, a suppressed immune system or a history of lung disease, you should avoid places where the aspergillus fungus is likely to be, such as:

  • marshland and bogs,
  • forests,
  • compost heaps,
  • grain stores,
  • rotting vegetation, and
  • piles of dead leaves.

The harmful effects of the fungus in buildings can be reduced by ensuring that there is good ventilation and dust control. This is particularly important if construction or renovation work is being carried out.

Keeping the room disinfected, maintaining room temperature and ensuring adequate air flow are all useful ways of preventing an aspergillosis infection.

Glossary

Immune system
The immune system is the body's defence system, which helps protect it from disease, bacteria and viruses.
Lung
Lungs are a pair of organs in the chest that control breathing. They remove carbon dioxide from the blood and replace it with oxygen.

Content provided by NHS Choices www.nhs.uk and adapted for Ireland by the Health A-Z.