Addison’s Disease

Addison’s disease is a rare but serious condition where your adrenal glands stop making enough vital hormones your body needs to function. Though it develops slowly and can be hard to spot at first, it’s manageable with daily medication and can allow you to live a normal life.

Table of contents

• What is Addison’s disease?
• Other names for this condition
• Parts of the body affected
• Who does it affect?
• Signs and symptoms
• What causes Addison’s disease?
• How is it diagnosed?
• Treatment and management
• Adrenal crisis: A medical emergency
• Living with Addison’s disease
• Related conditions

What is Addison’s disease?

Addison’s disease is a chronic condition in which your adrenal glands don’t produce enough of the hormones cortisol and aldosterone^[1]. Your adrenal glands, also known as suprarenal glands, are small, triangle-shaped glands that sit on top of each of your two kidneys. They’re part of your endocrine system, which is the network of glands that make hormones^[1].

Cortisol is a hormone that helps your body respond to stress, including the stress of illness, injury or surgery. It also helps maintain your blood pressure, heart function, immune system and blood glucose (sugar) levels. Cortisol is essential for life^[1].

Aldosterone is a hormone that affects the balance of sodium (salt) and potassium in your blood. This in turn controls the amount of fluid your kidneys remove as urine, which affects blood volume and blood pressure^[1].

Addison’s disease was named after Thomas Addison, the London doctor who first identified the condition around 1850^[7].

Other names for this condition

Primary adrenal insufficiency, Autoimmune adrenalitis, Adrenocortical hypofunction, Hypocortisolism

Parts of the body affected

• Adrenal glands
• Kidneys
• Pituitary gland
• Endocrine system

Who does it affect?

Addison’s disease is rare. In the United States, it affects 1 in 100,000 people^[1]. The condition can affect people of all age groups, but it’s most common in people 30 to 50 years old^[1][4].

People who have autoimmune polyendocrine syndrome, a rare, inherited condition in which your immune system mistakenly attacks many of your tissues and organs, are much more likely to have Addison’s disease. Your mucous membranes, adrenal glands and parathyroid glands are commonly affected by this syndrome, though it can affect other types of tissues and organs^[1].

People who have the following autoimmune diseases are also at higher risk of developing the autoimmune form of Addison’s disease^[1]:

• Type I diabetes
• Pernicious anemia
• Graves’ disease
• Chronic thyroiditis
• Dermatitis herpetiformis
• Vitiligo
• Myasthenia gravis

Up to 50% of patients with Addison’s disease develop another autoimmune disorder during their lifetime^[12].

Signs and symptoms

With Addison’s disease, the damage to your adrenal glands usually happens slowly over time, so symptoms occur gradually. The disease may happen so slowly that people who have it might ignore the symptoms at first^[2]. Symptoms vary from person to person^[1].

Symptoms of Addison’s disease include^[1][4]:

• Steadily worsening fatigue (most common symptom)
• Patches of dark skin (hyperpigmentation), especially around scars and skin creases and on your gums – this may be harder to see on brown or black skin
• Abdominal pain
• Nausea and vomiting
• Diarrhea
• Loss of appetite and unintentional weight loss
• Muscle pain, muscle spasms and/or joint pain
• Dehydration
• Low blood pressure, which can cause lightheadedness or dizziness upon standing
• Changes in mood and behavior, such as irritability, depression and poor concentration
• A craving for salty food
• Low blood sugar (hypoglycemia)
• Headache
• A slightly raised temperature
• Feeling thirsty and needing to pee a lot
• Difficulty concentrating
• Anxiety and depression

Women with Addison’s disease may also have abnormal menstruation (periods), lose body hair and have a decreased sexual drive^[1].

Physical stress such as an illness or injury can make symptoms get worse fast^[2].

What causes Addison’s disease?

Addison’s disease results from the destruction of the bilateral adrenal cortex, leading to decreased adrenocortical hormones, including cortisol, aldosterone, and androgens^[3].

The most common cause of primary adrenal insufficiency is Addison’s disease, which is associated with increased levels of 21-hydroxylase antibodies. Autoimmune adrenalitis is a disorder in which the adrenal cortex is destroyed by an autoimmune process^[3][12]. This means the body’s immune system mistakenly attacks its own tissues, damaging the adrenal glands^[5].

Other causes of Addison’s disease include^[3][6]:

• Infections such as tuberculosis
• Cancer that has spread to the adrenal glands
• Hemorrhage (bleeding in the adrenal glands)
• Certain medications like antifungal medicines
• Adrenoleukodystrophy
• Bleeding caused by blood-thinning medicine
• Injury to the gland from a blow to the back

As the disease develops, individuals lose adrenocortical function over a period of years. The production of 21-hydroxylase antibodies can precede symptom onset by years to decades, and they are present in more than 90% of recent-onset cases^[12].

How is it diagnosed?

Diagnosis involves steps that your healthcare team takes to find out if you have Addison’s disease. Your healthcare professional talks with you about your medical history and your symptoms^[8]. A high clinical suspicion should be maintained to avoid misdiagnosis^[3].

If a doctor thinks you or your child might have Addison’s disease, they’ll refer you to a specialist. This is usually a hormone specialist (endocrinologist), although children are often referred to a doctor who specializes in the treatment of children (paediatrician)^[4].

You might have some of the following tests^[8][18]:

• Blood test: This test can measure blood levels of sodium, potassium, cortisol and adrenocorticotropic hormone (ACTH). A blood test also can measure proteins called antibodies related to Addison’s disease caused by an autoimmune disease.
• ACTH stimulation test: ACTH tells the adrenal glands to make cortisol. This test measures the level of cortisol in the blood before and after a shot of lab-made ACTH. This is the primary test to confirm the diagnosis.
• Insulin-induced hypoglycemia test: This test is done to find out if the pituitary gland is causing secondary adrenal insufficiency. The test involves checking blood sugar and cortisol levels after a shot of insulin.
• Imaging tests: A CT scan of the stomach area checks the size of the adrenal glands and looks for other issues. An MRI of the pituitary gland can spot damage that may cause secondary adrenal insufficiency.
• Blood tests to check your hormone levels and how well your thyroid gland is working

The diagnosis is established by demonstrating low cortisol and aldosterone levels, high renin levels, and a blunt cortisol response with ACTH stimulation^[3].

Treatment and management

There’s currently no cure for Addison’s disease, but there are medicines that can help manage the condition^[4]. Addison’s disease treatment involves taking medicines to replace the missing hormones your body needs. You will need to take hormone pills for the rest of your life^[5].

The main treatment is steroid medicine to replace the missing hormones. Medicines you may be given include^[4][8]:

• Hydrocortisone (Cortef), prednisone (Rayos, Prednisone Intensol) or methylprednisolone (Medrol) to replace cortisol. You take these medicines on a schedule. This helps mimic the changes in cortisol levels the body typically goes through over 24 hours.
• Fludrocortisone acetate to replace aldosterone. You’ll likely need plenty of sodium in your diet.

You’ll usually need to take your medicine every day for the rest of your life. It’s important you always have enough to avoid running out^[4]. Treatment should be titrated to the lowest dose that relieves symptoms^[12].

At certain times you may need to take extra medicine, such as when you’re unwell, about to have surgery, or during stressful periods like when taking exams. Your specialist will be able to advise you about this^[4]. During times of major stress, trauma, or surgery, you’ll need higher “stress” doses of corticosteroids^[10].

Some women may benefit from DHEA (dehydroepiandrosterone) therapy, which may improve depression symptoms and health-related quality of life^[12].

If you’re taking steroids, you should carry a steroid emergency card with you at all times. This tells healthcare professionals you have Addison’s disease and you take steroid medicine that should not be stopped suddenly^[4]. You can also get jewellery and wristbands with details of your condition and the medicine you take engraved or printed on them^[4].

Adrenal crisis: A medical emergency

In some cases — such as after an injury or severe illness or time of intense stress — symptoms can come on quickly and cause a life-threatening event called an addisonian crisis or acute adrenal failure^[1].

Adrenal crisis is the most serious complication of Addison’s disease. During adrenal crisis your symptoms get worse very quickly. This can be triggered by things like an infection, an accident or injury, or surgery^[4]. An addisonian crisis is a medical emergency. If it’s not treated, it can lead to shock and death^[1].

Symptoms of an addisonian crisis include^[1][4]:

• Extreme weakness
• Sudden, severe pain in your lower back, belly or legs
• Feeling restless, confused or fearful
• Severe vomiting and diarrhea
• Extreme weakness or feeling that you are going to pass out
• A high fever
• A fast heart rate
• Feeling very dizzy or light-headed (particularly when standing up after sitting or lying down)
• Severe dehydration
• Low blood pressure (hypotension)
• Seizures (fits)
• Loss of consciousness

It can cause problems such as stroke or your heart suddenly stopping (cardiac arrest)^[4].

Call 999 or emergency services immediately if you have Addison’s disease and the symptoms suddenly get worse. Adrenal crisis can usually be treated with an injection of steroid medicine^[4]. You should be given an emergency kit so you can give the injection yourself if needed. Your specialist may also give you or your carer training in what to do if you have adrenal crisis^[4]. It’s important to call emergency services even if you’ve given yourself an emergency steroid injection^[4].

Treatment for adrenal crisis includes immediate IV injections of corticosteroids and large amounts of IV saline, a salt solution, with dextrose added^[10].

Living with Addison’s disease

Addison’s disease is a serious condition that can be challenging to live with. You’ll need to remember to take your steroid medicine every day and there may be times when your symptoms get worse or you need emergency treatment^[4].

But with treatment, you can have a good quality of life with a normal life expectancy. It is not unknown for people with Addison’s to live into their 90s^[7]. With the right balance of daily medication, people with Addison’s and adrenal insufficiency can expect to have a normal life span^[7].

Here are some important steps you can take to care for yourself^[16]:

• Take your medicines exactly as prescribed. You will have to take medicines for the rest of your life.
• Wear medical alert jewellery. This lets others know that you have Addison’s disease.
• Have a shot of emergency medicine with you at all times. Know when and how to give yourself the medicine.
• Weigh yourself regularly, especially if you haven’t felt like eating or you’ve been vomiting.
• Keep track of your blood pressure. Let your doctor know if it’s high or too low.
• Work with your doctor to create a plan for what to do when you’re sick or when your body is under stress. You may need to change the amount of medicine you take during this time.
• Don’t reduce salt in your diet. You may need to add extra salt to your food during hot and humid weather or after exercise to replace salt lost through sweating.

Follow-up care is a key part of your treatment and safety. Be sure to make and go to all appointments^[16]. You’ll have appointments with your healthcare team to monitor your condition^[4].

Physicians should remain vigilant for the development of concomitant autoimmune disorders in patients with Addison’s disease^[12].

Related conditions

Addison’s disease is also called primary adrenal insufficiency. A related disorder, secondary adrenal insufficiency, happens when your pituitary gland doesn’t release enough adrenocorticotropic hormone (ACTH), which activates your adrenal glands to produce cortisol^[1].

The opposite condition to Addison’s disease is Cushing’s syndrome, which happens when your body has too much cortisol (hypercortisolism)^[1].