Addison disease is a condition most often caused by autoimmune issues. Researchers say that rare cases may be linked to genetics, which means the condition may run in families.

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Addison disease, also called “primary adrenal insufficiency,” is a serious condition in which the adrenal glands don’t produce essential hormones in the body. Without treatment, this condition may lead to fatigue, weight loss, muscle weakness, and other symptoms.

Read on to find out what researchers are saying and more about what causes this rare condition.

Here’s more information about Addison disease.

The adrenal glands are located above the kidneys. They produce the hormones cortisol and aldosterone. These hormones play important roles throughout the body in the tissues and organs. Most cases of Addison disease are caused by damage to the adrenal glands from a variety of factors.

Experts suspect that, in rare cases, there may be a genetic component to Addison disease. One link may be between certain genetic disorders that damage the adrenal glands.

The disorders — such as congenital adrenal hyperplasia and adrenoleukodystrophy — may affect the way the glands develop and how they function.

Additionally, a 2021 study recently identified nine gene variations linked to Addison disease. Of all variations, the AIRE gene may be specifically linked to the disease.

Before the 2021 study, the human leukocyte antigen complex (proteins found in the white blood cells) was the strongest genetic area related to an increased risk of Addison disease.

The other gene variations with a possible link to the disease include:

  • BACH2
  • PTPN22
  • CTLA4
  • LPP
  • SH2B3

More research is needed to fully understand the role these genes play in the development of Addison disease.

Damage to the adrenal glands in Addison disease is primarily caused by autoimmune issues. In fact, in between 8 and 9 out of 10 people with Addison disease, the condition may be related to autoimmune issues.

Other causes may include:

Language matters

You’ll notice we use the binary terms “women” and “men” in this article. While we realize these terms may not match every person’s gender experience, they’re the terms used by the researchers whose data was cited. We try to be as specific as possible when reporting on research participants and clinical findings.

Unfortunately, the studies and surveys referenced in this article didn’t report data for or may not have had participants who are transgender, nonbinary, gender nonconforming, genderqueer, agender, or genderless.

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Addison disease is rare. Only between 4 and 11 people per 100,000 are affected. Women are more likely to develop the disease than men, and the usual age when it starts is between 30 and 50 years.

Addison disease is often caused by autoimmune issues. The risk of developing it increases if a person has another autoimmune disease, including:

There’s no cure for Addison disease. It can be treated with medications to lessen inflammation (corticosteroids) and hormone replacements to restore the hormones that the adrenal glands aren’t producing.

These medications may replace hormones such as cortisol or aldosterone. For example, cortisol can be replaced with hydrocortisone, prednisolone, or dexamethasone, and aldosterone can be replaced with fludrocortisone.

Without treatment, a person may experience an Addisonian crisis marked by symptoms such as weakness, dizziness, and vomiting. This acute adrenal crisis is a life threatening situation and could lead to seizures, coma, or death if not treated promptly.

Addison disease requires ongoing monitoring and treatment. That said, most people live relatively average lives and don’t have restrictions on what activities they can enjoy.

People who have Addison disease are also at an increased risk of developing other autoimmune conditions. Likewise, treatment itself may lead to additional health difficulties, such as obesity, diabetes, osteoporosis, or hypertension.

Can Addison disease run in families?

To “run in families,” a disease must be in genes that are passed during conception. Researchers believe that, in rare circumstances, Addison disease may run in families through inherited autoimmune or genetic disorders.

What triggers Addison disease?

Autoimmune diseases trigger Addison disease in 80–90% of cases. The cause of autoimmune issues is unknown but may be related to genetics, infections, and environmental factors.

Is Addison disease caused by stress?

Addison disease isn’t caused by stress. Some people believe stress may cause something called “adrenal fatigue.” This isn’t a true diagnosis of any existing condition.

While rare, you may be at a higher risk of developing Addison disease if other people in your family have this condition.

Make an appointment with a doctor if you experience symptoms that concern you, such as fatigue, discolored patches on your skin, or gastrointestinal symptoms. A doctor can run the necessary tests to accurately diagnose your condition and create a treatment plan to improve your quality of life.