Addison's disease (or Addison disease) is a rare condition in which your body doesn't make enough of two hormones: cortisol and aldosterone. There are five stages of progression in Addison's disease.
Addison’s disease (or Addison disease) is a rare condition in which your body doesn’t make enough of two hormones: cortisol and aldosterone. There are five stages of progression in Addison's disease.

Addison’s disease (or Addison disease) is a rare condition in which your body doesn’t make enough of two hormones: cortisol and aldosterone. It's also known as primary adrenal insufficiency.

Causes of Addison’s disease

Addison’s disease is caused by damage to the outermost layers of your adrenal glands. This is where your body makes the hormones aldosterone and cortisol.

Cortisol helps your body respond to stress. It also has several other important body functions:

Aldosterone affects your body’s balance of sodium and potassium. If you don’t have enough aldosterone, it lowers your kidney’s ability to filter salt and water, resulting in low blood pressure.

In about 75% of people with Addison’s disease, this damage to the adrenal glands is due to an autoimmune reaction. This is when your body’s immune system wrongly attacks healthy tissue.

Tuberculosis used to be a major cause of Addison’s disease. This infectious disease can damage your adrenal glands. But this is a less common cause in developed countries once tuberculosis treatment improved.

Other causes of Addison’s disease include:

  • Bleeding in your adrenal glands
  • Cancer cells in your adrenal glands
  • Genetic disorders that affect how your adrenal glands function or develop
  • Adrenal gland removal
  • Some medications like antifungal drugs

Addison’s disease stages

There are five stages of progression in Addison's disease:

  • Stage 0: Normal adrenal function. 
  • Stage 1: You may have increased plasma renin with normal or low levels of aldosterone but no other abnormal results. 
  • Stage 2: You're likely to have high renin levels and low aldosterone levels. Renin is an enzyme produced in your kidney. It helps regulate your blood pressure. With an ACTH stimulation test, your cortisol levels may have low or no response.
  • Stage 3: Your ACTH levels increase, and you may have normal or low cortisol levels. ACTH or adrenocorticotropic hormone is made by your pituitary gland. It stimulates your adrenal gland to produce cortisol.
  • Stage 4: There are very high levels of ACTH and low cortisol levels. You may also have symptoms of Addison’s disease.

Addisonian crisis

If you have Addison’s disease, but it’s undiagnosed and untreated, an Addisonian crisis (adrenal crisis) can happen. This is usually triggered by events of high stress such as trauma, accident, severe infection, or surgery. An Addisonian crisis is a medical emergency. 

Most cases of Addisonian crisis are in people with Addison’s disease who experience a high-stress event or who haven’t been taking their replacement hormone drugs. In a study of 444 people with Addison’s disease, 42% reported at least one Addisonian crisis.

Symptoms of an Addisonian crisis include:

If not treated, an Addisonian crisis can lead to severe complications like:

Symptoms of Addison’s disease

Addison’s disease symptoms progress slowly and tend to be nonspecific. So it’s often confused with other illnesses.

Early-stage Addison’s disease symptoms include:

Other symptoms of Addison’s disease include:

Treatment for Addison’s disease

Treatment for Addison's disease is medication. You'll need to take hormone drugs to replace what your body can't produce. The drug fludrocortisone replaces aldosterone, and cortisol is replaced by hydrocortisone.

If you have an Addisonian crisis, you’ll need immediate medical help. You’ll be injected with a high dose of hydrocortisone. You may also be given fluids, electrolytes, and vasopressors to help maintain your blood pressure.

Prevention

You can’t prevent Addison’s disease, but you can take some steps to avoid an adrenal crisis:

  • If you have Addison’s disease, always wear a medical alert wristband or carry a card stating you have the disease.
  • Educate yourself, your family, and caregivers of the symptoms of an Addisonian crisis. Get immediate medical help if you develop these symptoms.
  • Carry an emergency vial of injectable hydrocortisone with you. Make sure that you and your family members are trained to administer this injection.

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Medically Reviewed on 1/12/2022
References

American Family Physician: “Addison Disease: Early Detection and Treatment Principles.”

Clinical Chemistry and Laboratory Medicine: "The natural history of autoimmune Addison’s disease with a non-classical presentation: a case report and review of literature."

Journal of Internal Medicine: “Clues for early detection of autoimmune Addison's disease – myths and realities.”

Medscape: “Addison Disease Clinical Presentation.”

Merck Manual: “Kidneys.”

National Institute of Diabetes and Digestive and Kidney Diseases: “Symptoms and Causes of Adrenal Insufficiency & Addison's Disease.”

National Organization for Rare Disorders: “Addison’s Disease.”

Rathbun, K.M., Nguyen, M., Singhal, M., StatPearls, “Addisonian Crisis,” StatPearls Publishing, 2021.

Therapeutic Advances in Endocrinology and Metabolism: “Adrenal crisis: prevention and management in adult patients.”

UCLA Health: “ACTH Stimulation Test."