Adrenal insufficiency

Also known as Addison's disease

Adrenal insufficiency is a condition that occurs when the adrenal glands do not produce enough of certain hormones. It can affect people of all ages. With treatment, people can live full and normal lives.

What is adrenal insufficiency? 

Adrenal insufficiency is an endocrine, or hormonal, disorder that occurs when the adrenal glands do not produce enough of certain hormones. Located just above the kidneys, the adrenal glands produce a range of important hormones including cortisol, aldosterone, adrenaline and androgens. 

Adrenal insufficiency can be primary (about 25%) or secondary (75% of cases):

  1. Primary adrenal insufficiency is also known as Addison's disease (after the doctor who described this condition). Addison’s disease is relatively rare affecting 110 to 144 of every 1 million people in developed countries.(1)  It occurs when the adrenal glands are damaged (from infection, injury, autoimmune problems etc) and cannot produce enough of the adrenal hormones cortisol and sometimes aldosterone.
  2. Secondary adrenal insufficiency is more common than Addison's disease and occurs when the pituitary gland (a pea-sized gland at the base of the brain) doesn't produce enough adrenocorticotropin (ACTH), which travels via the blood stream to stimulate the adrenal glands to produce the hormone cortisol. Over time, without enough ACTH, the adrenal glands can shrink. 

Adrenal insufficiency can affect both males and females of any age.

Symptoms of adrenal insufficiency

The symptoms of adrenal insufficiency are often vague and non-specific. They often come on gradually, first appearing during times of stress and then more often over weeks to months. Such symptoms include:

  • abdominal pain
  • fatigue
  • muscle weakness
  • low blood pressure that drops further when a person stands up, causing dizziness or fainting
  • diarrhoea or constipation
  • nausea and /or vomiting 
  • weight loss
  • joint and muscle aches
  • headache
  • hypoglycaemia
  • sweating
  • hyperpigmentation (only in Addison’s disease – dark patches or skin discolouration, especially in the folds of the skin or around areas such as the nipples, lips, and rectum)
  • dehydration (only in Addison’s disease)
  • salt craving (only in Addison’s disease)
  • irritability and depression
  • irregular or absent menstrual periods
  • in women, loss of interest in sex.

Causes of adrenal insufficiency

Addison's disease

The most common cause of Addison’s disease (in 80% of cases) is an autoimmune disorder. Autoimmune disorders occur when the body’s immune system attacks the body’s own cells and organs. In autoimmune Addison’s, (most common in middle age women) the immune system gradually destroys the outer layer of the adrenal glands, known as the adrenal cortex.

Other causes include:(2) 

  • viral (cytomegalovirus), bacterial (tuberculosis, Neisseria meningitidis) and fungal infections
  • tumours
  • medications
  • genetic defects
  • amyloidosis.

Secondary adrenal insufficiency

Anything that affects the pituitary gland can result in less pituitary hormones reaching the adrenal glands to tell them to produce their hormones. This can include pituitary tumours, surgical removal or damage to the pituitary gland, damage to the blood supply and medication related.

A temporary form of secondary adrenal insufficiency can occur when someone has been on corticosteroids such as Prednisone for weeks or months to treat inflammatory illnesses such as rheumatoid arthritis, asthma, and ulcerative colitis. Taking corticosteroids for prolonged periods results in the adrenal glands producing less of their natural hormones and when the prescription doses are stopped, the adrenal glands may be slow to restart. This is why it is very important that prescription corticosteroid doses are reduced gradually over a period of weeks or even months to reduce this occurring.

Diagnosis & tests

It can be hard to diagnose adrenal insufficiency especially when the disease is still in its early stages. Your doctor may be able to suspect it after examining your symptoms and medical history.

Diagnosis is confirmed through urine and hormonal blood tests. A doctor uses the results of these tests to determine whether the levels of cortisol are too low. Next, your doctor will want to work out whether one has primary or secondary adrenal insufficiency and what the underlying cause is. Imaging studies of the pituitary and adrenal glands may be needed. 

Treatment of adrenal insufficiency

Adrenal insufficiency is treated using medicines which aim to replace the missing hormones. Depending on the cause, you may need to take these medicines for the rest of your life. With treatment, it is possible to largely control the symptoms of the disorder and live a full and active life. 

Self care – managing an adrenal crisis

The most thing you can do to look after yourself is to make sure you understand what an adrenal crisis is and know what to do if you have one.

An adrenal crisis can happen when cortisol levels in your body fall significantly, resulting in sudden, severe symptoms (more information below). It is normally treated using adrenal hormones and you will be given tablets to have on hand at all times.

You also need to carry a corticosteroid injection with you at all times and ensure that other people in your company know how and when to administer this, should you become so unwell you are unable to do it yourself. 

Possible complications – adrenal crisis

Taking too much or too little adrenal hormone replacement can lead to a number of hormone related complications. However, the most serious complication is the risk of an adrenal crisis.

Adrenal crisis

An adrenal crisis – or for people with Addison’s disease, an Addisonian crisis –is a life-threatening event that can occur when the body is not able to produce enough stress hormones to cope with a stressful event such as infection, injury or surgery. 

Symptoms of an adrenal crisis include:(2)

  • sudden, severe pain in the lower back, abdomen, or legs
  • severe vomiting and diarrhoea
  • dehydration
  • low blood pressure
  • loss of consciousness.

If not treated, an adrenal crisis can cause death. 

Once someone knows they have adrenal insufficiency, they are given medication to take if such events occur and intravenous corticosteroids are given during and after surgery. 

When to seek medical help

Contact your healthcare provider if: 

  • Your ankles start swelling.
  • You can’t keep your medication down as a result of vomiting.
  • You notice new symptoms.
  • Your weight changes without reason.
  • You have stress like infection, trauma, injury or dehydration (straight away).

Learn more

Addison's disease NHS Choices, UK
Addison's disease leaflet Patient Info, UK

References

  1. Betterle C, Morlin L. Autoimmune Addison’s disease. In: Ghizzoni L, Cappa M, Chrousos G, Loche S, Maghnie M, eds. Pediatric Adrenal Diseases. Endocrine Development. Vol. 20. Padova, Italy: Karger Publishers; 2011: 161–172
  2. Adrenal insufficiency and Addison's disease National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), USA
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