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Adrenal Insufficiency

January 24, 2022

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Primary adrenal insufficiency (AI), also called Addison's disease, is a rare condition where the adrenal glands do not work properly and cannot make enough cortisol (a “stress” hormone). Usually, production of aldosterone and androgens (the two other types of steroid hormones made by the adrenal glands) is also low. 

Secondary AI is the far more common type of AI. It results when the pituitary gland, a small gland near the brain, does not signal the adrenal glands to make cortisol. Production of aldosterone is normal in secondary AI.  

The adrenal glands do not get “adrenal fatigue” or lose function because of mental or physical stress. True AI is a rare health problem. Only an endocrinologist, an expert in hormones, should diagnose AI using standard tests. 

Endocrine Connection 

The adrenal glands, located on top of the kidneys, make hormones that are essential for body functions. The outer layer (cortex) of the adrenal glands makes three types of steroid hormones. In AI, the adrenal cortex does not make enough steroid hormones.  

Primary AI: The most common cause of primary AI is autoimmune disease, meaning the body's defense system attacks and destroys the body's own tissues. When adrenal glands are damaged, they can't produce hormones. Other causes of primary AI include bleeding in the glands, infections, genetic (inherited) diseases, and surgical removal of the adrenal glands. 

Secondary AI: Problems with the pituitary gland cause secondary AI. Normally, the pituitary gland makes a hormone called ACTH, which tells the adrenal glands to make cortisol. But in secondary AI, the pituitary gland does not send enough ACTH to the adrenal glands. Therefore, not enough cortisol is made. 

Some causes of secondary AI may be temporary, such as taking certain prescription medicines like prednisone, hydrocortisone, or dexamethasone. Other causes may be permanent, such as pituitary tumors, pituitary surgery, or radiation damage to the pituitary. 

If your doctor is concerned about AI after reviewing your symptoms and medical history, they may check blood levels of cortisol and other hormones, as well as electrolytes such as sodium and potassium, to detect AI and help find the cause. They may also look at the adrenal glands or the pituitary gland with imaging tests, such as CT or MRI scans.

Symptoms of AI include fatigue, muscle weakness, decreased appetite, and weight loss. Some people experience lightheadedness, dizziness, nausea, vomiting, abdominal pain, and diarrhea. Other symptoms include: 

  • Pain in the muscles and joints 
  • Low blood pressure  
  • Cravings for salt (in primary AI) 
  • Symptoms of low blood glucose, such as sweating (more common in children with AI)  
  • Darkened skin on the face, neck, and back of the hands (in primary AI) 
  • Irregular menstrual periods in women 

Some people don't know they have AI until they have a sudden worsening of symptoms called an adrenal crisis. 

Physical stress caused by illness, infection, surgery, or an accident can suddenly make symptoms of AI much worse, an emergency illness called an adrenal crisis. If left untreated, adrenal crisis can cause death. Adrenal crisis occurs mainly in people with primary AI. 

People in adrenal crisis need an injection (shot) of glucocorticoids (medicines that replace cortisol) right away. Then they need to go to the hospital immediately for more treatment. If you have AI, you should wear a medical alert bracelet or tag and know the warning signs of adrenal crisis. They include: 

  • Severe nausea and vomiting 
  • Diarrhea 
  • Abdominal pain 
  • Dehydration and confusion 
  • Low blood pressure and fainting

The goal of treatment is to ensure proper hormone levels day-to-day. You may need daily replacement of hormones for life. You will take glucocorticoids to replace the cortisol your body no longer makes. You may also need mineralocorticoids if your body does not make aldosterone (a hormone that regulates blood pressure). 

Extra glucocorticoids may be needed during times of stress, such as serious illness or surgery. Your doctor will provide personalized advice on adjusting medicines for stress. Understanding your disease and knowing when and how to adjust your medications can help you live a long and healthy life with AI.  

  • What type of AI do I have? 
  • Is my AI temporary or permanent? 
  • Will I need hormone replacement? 
  • If I am in adrenal crisis, how do I give myself a glucocorticoid shot? 

Endocrine Library >>

Editor(s): Baha Arafah, M.D., Richard Auchus, M.D., Irina Bancos, M.D., Melanie Schorr Haines, M.D.
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