Addison’s disease
Addison’s disease
About Addison's disease
Symptoms of Addison's disease
Causes of Addison's disease
Diagnosing Addison's disease
Treating Addison's disease
About Addison's disease
Addison's disease, also called primary adrenal insufficiency or hypoadrenalism, is a rare disorder affecting the adrenal glands. If you or someone in your family has just been told about this condition, it can feel unfamiliar and worrying, so let's go through it calmly.
You have 2 small adrenal glands, one sitting on top of each kidney. These glands make hormones your body depends on every day, including cortisol, aldosterone and adrenaline. In Addison's disease, the adrenal gland becomes damaged, so it can no longer make enough cortisol and aldosterone.
This is a rare condition. Around 8,400 people in the UK live with Addison's disease, and it can appear at any age, though it shows up most often between the ages of 30 and 50. It is also seen more frequently in women than in men.
In its early stages, Addison's disease can feel a lot like other, more common problems such as depression or flu, which is part of why it is easy to overlook at first. You might notice:
fatigue, a persistent lack of energy or motivation
muscle weakness
low mood
loss of appetite along with weight loss you did not intend
increased thirst
As time goes on, these symptoms can become more pronounced, and you may also experience dizziness, fainting, cramps and a deep sense of exhaustion. Some people notice small patches of darkened skin, or darker lips or gums.
These symptoms do not always mean Addison's disease is the cause, but it is still worth having them checked by your doctor so the reason can be properly investigated. You can read more about the symptoms of Addison's disease and how it is diagnosed.
Information about you
If you are diagnosed with Addison's disease, your clinical team will share some of your information with the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS). This helps researchers find better ways to prevent and treat the condition over time. If you would rather not be included, you can opt out of the register whenever you choose. You can find out more about the register if you would like further details.
Common Symptoms
⚠️ This symptom list is for educational purposes. Having these symptoms does not mean you have this condition. See a doctor for proper diagnosis.
Medicines Commonly Used
⚠️ These medicines are commonly used for confirmed diagnoses of this condition. This is NOT a recommendation or prescription. Treatment must be determined by your doctor.
No medicines have been linked to this condition yet.
When to See a Doctor
You and a partner or family member can be trained to give a hydrocortisone injection in an emergency.
This might be necessary if you go into shock after an injury, or if vomiting or diarrhoea keep you from keeping oral medicine down. This can happen if you are pregnant and dealing with morning sickness. Your endocrinologist will talk with you about when such an injection might be needed.
If you ever have to give yourself emergency hydrocortisone, phone your GP right afterwards. It also helps to know what out of hours services are available in your area, in case the emergency happens outside normal working hours.
You can register with your local ambulance service as well, so they keep a record that you need a steroid injection or tablets if you ever require their help.
Causes
Addison's disease usually develops because the immune system turns against the body's own tissue, attacking the outer layer of the adrenal gland (the adrenal cortex) and disrupting its production of two essential steroid hormones, aldosterone and cortisol. Doctors don't fully understand why this happens, but in the UK it accounts for 70% to 90% of cases.
Other causes include illnesses that damage the adrenal glands directly, such as tuberculosis (TB), though this is uncommon in the UK.
Read more about the causes of Addison's disease
Causes of Addison's disease
Addison's disease develops when the outer layer of your adrenal glands, known as the adrenal cortex, becomes damaged and produces lower levels of hormones than your body needs.
Problems with the immune system
In the UK, the most common cause of Addison's disease is a problem with the immune system, responsible for 70% to 90% of cases.
Your immune system is normally your body's defence against infection and disease. When you fall ill, it produces antibodies, special proteins that destroy disease-causing organisms and toxins, to fight off the threat.
Sometimes, though, something goes wrong with this system, and it starts attacking your own healthy tissues and organs instead. This is called an autoimmune disorder.
Addison's disease can develop when your immune system mistakenly attacks your adrenal glands and severely damages the adrenal cortex. Once about 90% of the adrenal cortex has been destroyed, your adrenal glands can no longer make enough cortisol and aldosterone. As levels of these hormones fall, the symptoms of Addison's disease begin to appear.
It isn't clear why some people develop this immune system problem in the first place, though it does seem to run in families.
Genetics
Research suggests that certain genes make some people more likely to develop autoimmune disorders.
Scientists don't yet know exactly how these genes lead to Addison's disease and similar conditions, but having one does raise your risk, especially if you or a close family member already has another autoimmune condition, such as:
- vitiligo, a long-term condition that causes pale, white patches to appear on the skin
- type 1 diabetes, a long-term condition caused by too much glucose in the blood
- an underactive thyroid gland (hypothyroidism)
Other causes
Worldwide, tuberculosis (TB) is actually the most common cause of Addison's disease, though it remains rare in the UK. TB is a bacterial infection that mainly affects the lungs but can spread to other parts of the body, and it can trigger Addison's disease if it damages the adrenal glands.
Other possible causes include:
- infections, such as those linked to AIDS or certain fungal infections
- a haemorrhage, meaning very heavy bleeding into the adrenal glands, sometimes associated with meningitis or other severe cases of sepsis
- cancer, if cancer cells from elsewhere in the body spread to the adrenal glands
- amyloidosis, a disease in which a protein called amyloid, produced by bone marrow cells, builds up in the adrenal glands and damages them
- surgical removal of both adrenal glands (adrenalectomy), for example to remove a tumour
- adrenoleukodystrophy (ALD), a rare, life-limiting inherited condition that affects the adrenal glands and nerve cells in the brain, most often in young boys
- certain treatments needed for Cushing's syndrome, a collection of symptoms caused by very high levels of cortisol in the body
Diagnosis
Diagnosing Addison’s disease
To help diagnose Addison’s disease, your GP will first ask about your symptoms and review your medical history.
They’re also likely to ask if anyone in your family has an autoimmune disorder (a condition caused by a problem with their immune system).
Your GP will examine your skin for any evidence of brownish discolouration (hyperpigmentation), particularly in certain areas, such as:
- where skin creases on your palm
- in your elbow crease
- on any scars
- your lips and gums
However, hyperpigmentation doesn’t occur in all cases of Addison’s disease.
You’ll also be tested for low blood pressure (hypotension) while you’re lying down and again shortly after you stand up. This is to see whether you have postural or orthostatic hypotension (low blood pressure when you change position).
Blood tests
If Addison’s disease is suspected, blood tests will be carried out to measure the levels of sodium, potassium and cortisol in your body. A low sodium, high potassium or low cortisol level may indicate Addison’s disease.
You may need to see a hospital hormone specialist (endocrinologist) for your blood to be tested for the following:
- a low level of the hormone aldosterone
- a high level of adrenocorticotrophic hormone (ACTH)
- a low level of glucose (sugar used for energy)
- positive adrenal antibodies (antibodies designed to attack the adrenal gland)
Any of these could be a sign of Addison’s disease.
Synacthen stimulation test
If cortisol in your blood is low, or your symptoms strongly suggest Addison’s disease, you’ll need to have a synacthen stimulation test to confirm the diagnosis.
Your GP may refer you to an endocrinology unit (a unit that specialises in the study of hormones) for the test. How urgently you’re referred depends on how severe your symptoms are.
Synacthen is a man-made (synthetic) copy of the adrenocorticotrophic hormone (ACTH). ACTH is naturally produced by the pituitary gland (a pea-sized gland below the brain) to encourage the adrenal glands to release the hormones cortisol and aldosterone.
When synacthen is administered, the adrenal glands should respond in the same way as they would to ACTH, and release cortisol and other steroid hormones into the blood.
A blood sample will be taken and tested for cortisol, before an injection of synacthen is given into your arm. After 30 and 60 minutes, a further blood sample will be taken for cortisol measurement.
If the ACTH level is high, but the cortisol and aldosterone levels are low, it’s usually confirmation of Addison’s disease.
Thyroid function test
As well as a synacthen stimulation test, your thyroid gland may also be tested to see if it’s working properly.
Your thyroid gland is found in your neck. It produces hormones that control your body’s growth and metabolism.
People with Addison’s disease often have an underactive thyroid gland (hypothyroidism), where the thyroid gland does not produce enough hormones. By testing the levels of certain hormones in your blood, your endocrinologist (a specialist in hormone conditions) can determine whether you have hypothyroidism.
Scans
In some cases, your specialist may refer you for a scan of your adrenal glands. This could be a computerised tomography (CT) scan or a magnetic resonance imaging (MRI) scan.
Diagnosis during an adrenal crisis
If Addison’s disease is left untreated, it eventually leads to an adrenal crisis. This is where the symptoms of Addison’s disease appear quickly and severely.
During an adrenal crisis, there’s not enough time to perform a synacthen stimulation test to confirm Addison’s disease.
If possible, blood will be taken and tested for any of the abnormalities listed above. While you’re waiting for the results, treatment may be started with steroid injections, and fluids containing salt and glucose.
Read more about treating Addison’s disease
Driving
If you’re diagnosed with Addison’s disease and have a bus, coach or lorry licence, it’s your legal obligation to inform the Driver and Vehicle Licensing Agency (DVLA).
Read about driving and Addison’s disease on the GOV.UK website.
Medication for Addison’s disease
Treatment usually involves corticosteroid (steroid) replacement therapy for life. Corticosteroid medication is used to replace the hormones cortisol and aldosterone that your body no longer produces. It’s usually taken in tablet form 2 or 3 times a day.
In most cases, a medication called hydrocortisone is used to replace the cortisol. Other possible medicines are prednisolone or dexamethasone, though these are less commonly used.
Aldosterone is replaced with a medication called fludrocortisone. Your GP may also ask you to add extra salt to your daily diet, although if you’re taking enough fludrocortisone medicine this may not be necessary. Unlike most people, if you feel the urge to eat something salty, then you should eat it.
In general, the medications used for Addison’s disease don’t have side effects, unless your dose is too high. If you take a higher dose than necessary for a long time, there’s a risk of problems such as weakened bones (osteoporosis), mood swings and difficulty sleeping (insomnia).
Treatment
Treating Addison’s disease
Addison’s disease is treated with medication to replace the missing hormones. You’ll need to take the medication for the rest of your life.
With treatment, symptoms of Addison’s disease can largely be controlled. Most people with the condition live a normal lifespan and are able to live an active life, with few limitations.
However, many people with Addison’s disease also find they must learn to manage bouts of fatigue and there may be associated health conditions, such as diabetes or an underactive thyroid.
People with Addison’s disease must be aware of the risk of a sudden worsening of symptoms, called an adrenal crisis. This can happen when the levels of cortisol in your body fall significantly due to not taking your medicines, or during another illness.
An adrenal crisis is a medical emergency and warning signs include severe nausea, confusion, fever, headache and weakness. If left untreated, it can be fatal. If you or someone you know has Addison’s disease and is experiencing severe symptoms, phone 999 for an ambulance.
Read more about treating Addison’s disease
If you have Addison’s disease, you’ll need to take daily medication to replace the lost hormones. This should help you to live an active life, although many people find they still need to manage their fatigue.
In some cases, the underlying causes of Addison’s disease can be treated. For example, tuberculosis (TB) is treated with a course of antibiotics over a period of at least 6 months.
However, most cases are caused by a problem with the immune system that can’t be cured.
Treating adrenal crisis
Adrenal crisis, or Addisonian crisis, needs urgent medical attention. Phone 999 for an ambulance if you or someone you know are experiencing adrenal crisis.
Signs of an adrenal crisis include:
- severe dehydration
- pale, cold, clammy skin
- sweating
- rapid, shallow breathing
- dizziness
- severe vomiting and diarrhoea
- severe muscle weakness
- headache
- severe drowsiness or loss of consciousness
In hospital, you’ll be given lots of fluid through a vein in your arm to rehydrate you. This will contain a mixture of salts and sugars (sodium, glucose and dextrose) to replace those that your body is lacking. You’ll also be injected with hydrocortisone to replace the missing cortisol hormone.
Any underlying causes of the adrenal crisis, such as an infection, will also be treated.
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Complications
Living with Addison's disease and adjusting your medication
Most people with Addison's disease find that once they're on the right medication, they can carry on with their usual diet and exercise routine. Still, tired spells are common, and it often takes a while to learn how to handle these low-energy periods.
Some people feel that having to take medication on a strict schedule is restrictive, and that it weighs on their daily life or emotional wellbeing. Missing a dose, or taking it late, can bring on exhaustion or trouble sleeping.
A number of people go on to develop related health conditions, such as diabetes or an underactive thyroid, which then need their own treatment and care.
You'll usually see an endocrinologist every 6 to 12 months so they can check your progress and adjust your medication dose if needed. Between these visits, your GP can offer support and issue repeat prescriptions.
If you don't take your medication as prescribed, you risk a serious condition called an adrenal crisis. To avoid this, you should:
- remember to collect your repeat prescriptions
- keep spare medication in useful places, such as your car or workplace, and always carry some with you
- take your medication at the same time every day, without fail
- pack extra medication when travelling, usually double what you'd normally need, along with your injection kit (covered below)
- carry your medication in your hand luggage when flying, along with a note from your doctor explaining why it's needed
It's also worth letting close friends or colleagues know about your condition. Explain the signs of an adrenal crisis to them, and what they should do if you have one.
Medical alert bracelets
Wearing a medical alert bracelet or necklace that shows you have Addison's disease is a wise precaution.
After a serious accident, such as a car crash, a healthy body produces extra cortisol. This helps a person cope with the stress and physical strain that comes with serious injury. Since your body can't make cortisol on its own, you'll need a hydrocortisone injection to replace it and prevent an adrenal crisis.
A medical alert bracelet lets any medical staff treating you know about your condition and the medication you need.
These bracelets and necklaces are pieces of jewellery engraved with your medical condition and an emergency contact number, and they're available from various retailers. Ask your GP if they can recommend one, or check the MedicAlert website.
If you're admitted to hospital, the staff looking after you also need to know that you require steroid replacement medication throughout your stay. This still applies even if you've been told not to eat or drink anything (nil by mouth) for any reason.
Adjusting your medication
There will be times when your medication needs adjusting to account for extra strain on your body. For instance, you may need a higher dose if you experience any of the following:
- an illness or infection, particularly one with a high temperature of 37.5C (99.5F) or above
- an accident, such as a car crash
- an operation, or a dental or medical procedure, such as a tooth filling or endoscopy
- strenuous exercise that isn't part of your usual daily routine
This helps your body cope with the added stress. Your endocrinologist will keep an eye on your dosage and advise on any changes needed. Over time, as you become familiar with the condition and learn what tends to trigger your symptoms, you may find you're able to adjust your own medication. Even so, always check with your GP or specialist whenever you're unsure.