Cause

Primary adrenal insufficiency (PAI) also known as Addison’s Disease is a life‐long condition, which must be controlled with daily steroids to replace hormones. If this medication is taken correctly and consistently, those with adrenal insufficiency (AI) can live full and long lives. Those with adrenal insufficiency should have regular medical support from those familiar with the condition, particularly endocrinologists – specialists in hormone‐related conditions.

Symptoms

The adrenal glands, found on top of the kidneys, produce hormones to maintain the functions of the body. These hormones are cortisol, aldosterone and DHEA. A person whose adrenal glands do not produce these hormones has primary adrenal insufficiency, or Addison’s Disease.

This rare condition is often autoimmune, meaning the body’s immune system makes a mistake and attacks the cells of the adrenal glands. If testing reveals that this is not the cause of a person’s adrenal insufficiency, there is a small probability that it is a sign of adrenoleukodystrophy (ALD) in males.

Testing for ALD at this stage is very important and is actively encouraged by Alex TLC.

While many boys and men with ALD or its adult form, adrenomyeloneuropathy (AMN) also have primary adrenal insufficiency, not all are affected by this.

ALD and AMN are rarely the cause of primary adrenal insufficiency, but it is important to test for this if the cause is not known (idiopathic Addison’s Disease).

Typical signs and symptoms of adrenal insufficiency are:

Medication Treatment

Occasionally there are drug shortages, Addison’s Disease Self-Help Group have an advice page on drug shortages that you may find helpful: Drug Shortage Causes and Coping Strategies for people with Addison’s Disease | Addison’s Disease Self-Help Group

Hydrocortisone sodium phosphate injection (the pre-mixed hydrocortisone injection) has been discontinued. Advanz Pharma confirmed in March 2026 that it has permanently discontinued the manufacture of hydrocortisone sodium phosphate in the UK. Addison’s Disease Self Help Group have created two informational videos showing how to give an emergency injection of Hydrocortisone 100mg Powder for Solution using a syringe in the event of an adrenal crisis. To find out more information: hydrocortisone sodium phosphate injection discontinued

Condition Management Information

Emergency Injection Kit

Alongside regular medication, those with adrenal insufficiency (Addison’s Disease) should carry an emergency injection kit in case of adrenal crisis. This sudden worsening of the condition, which can be brought on by stress or infection, can be life‐threatening. Emergency injections of hydrocortisone are required immediately to deal with this. This information is provided by kind permission of the Addison’s Disease Self Help Group.

Addison’s Self Help Group

For more information on managing adrenal insufficiency (Addison’s Disease), see the ADSHG free guidelines on Managing Your Addison’s and Adrenal Crisis Guidelines. To learn more about adrenal insufficiency, see the ADSHG website and consider becoming a member.

Society for Endocrinology’s Adrenal Crisis Information

Patients with adrenal insufficiency require regular glucocorticoid replacement and the daily dose requires adjustments during periods of ill health, fever, surgery. If this is not done, patients can suffer an adrenal crisis.

For more information and for the NHS Steroid Emergency Card, view this adrendal crisis information on the Society for Endocrinology website.

Informational Videos

Below are the links to two short video infographics on Primary Adrenal Insufficiency. These ‘CoMICs’ have been developed by University of Birmingham in collaboration with the ADHSG:

What is Addison’s disease

What is Adrenal crisis and how to manage it