Understanding Autoimmune Adrenalitis (Addison’s Disease)

Autoimmune adrenalitis is an autoimmune disease where your immune system attacks your adrenal glands. You have two adrenal glands. Each one is located on the top of your kidneys. They work to produce a variety of different hormones.

Autoimmune adrenalitis specifically affects the adrenal cortex, which is the outer part of the adrenal gland. The immune system attacks and destroys this area, resulting in the loss of the production of two hormones:

• Cortisol. One of the main hormones involved in your responses to stress, cortisol also has a role in reducing inflammation and regulating things like metabolism, blood sugar, and blood pressure.
• Aldosterone. Helping to maintain the balance of potassium and sodium in your blood, aldosterone is important for maintaining healthy blood pressure, stable heart rate, and proper function of nerves and muscles.

A lack of these hormones is what causes symptoms. Potentially life threatening complications can occur if the condition isn’t treated.

Autoimmune adrenalitis is the most common cause of primary adrenal insufficiency. The more common name for this condition is Addison’s disease.

The symptoms of autoimmune adrenalitis can come on slowly and be nonspecific. Because of this, they may sometimes be mistaken for other health conditions.

Many people with autoimmune adrenalitis won’t have symptoms until severe damage to the adrenal cortex has occurred. In fact, the National Organization for Rare Disorders estimates that symptoms don’t happen until about 90 percent of the adrenal cortex has been destroyed.

When they do appear, the symptoms of autoimmune adrenalitis can include:

• chronic fatigue
• weakness
• unexplained weight loss
• reduced appetite
• abdominal pain
• nausea
• vomiting
• diarrhea
• muscle and joint pain
• darkened areas of skin, called hyperpigmentation, in areas like the gums and lips, armpits, elbows, knuckles, and creases of the palms
• orthostatic hypotension, in which dizziness or fainting happens when you stand up from a sitting position
• low blood sugar, or hypoglycemia
• a craving for salty foods
• mood changes like irritability and depression

When to seek emergency care

People with autoimmune adrenalitis cannot effectively produce cortisol in response to stress. Because of this, a lack of cortisol in times of stress can lead to a life threatening complication called an adrenal crisis.

Go to the emergency room or call 911 if you or someone else has symptoms like:

• pain in your abdomen, lower back, or legs that comes on suddenly and is severe
• weakness
• vomiting
• diarrhea
• confusion
• loss of consciousness

The exact cause of autoimmune adrenalitis isn’t known. It’s generally believed that a combination of genetic and environmental factors contribute to the development of the condition.

Certain variations in genes for human leukocyte antigen (HLA) complex proteins are associated with autoimmune adrenalitis. HLA proteins play a vital role in the way your immune system distinguishes your own tissue from foreign invaders like bacteria and viruses.

In autoimmune adrenalitis, your immune system targets a normal protein called 21-hydroxylase, which is important for hormone production in the adrenal cortex. The resulting immune response to 21-hydroxylase causes damage to the adrenal cortex.

Autoimmune adrenalitis can be difficult to diagnose. This is due to the fact that symptoms can come on slowly and many early symptoms are vague or nonspecific.

First, your doctor will take your medical history. They’ll ask questions about your symptoms and whether or not you or other people in your family have a history of autoimmune disease.

Then, they’ll do a physical examination. During this time, they’ll check your vital signs and may check your skin for areas of hyperpigmentation.

Your doctor may also take a sample of your blood from your arm to use for blood tests if necessary.

After taking your medical history and doing a physical examination, your doctor can use the following tests to help diagnose autoimmune adrenalitis:

• Cortisol test. A cortisol test measures the levels of cortisol in your blood. The sample is taken in the morning. Low levels can indicate that your adrenal glands aren’t functioning well.
• Aldosterone test. An aldosterone test looks at levels of aldosterone in your blood. Similar to cortisol, low levels can point to adrenal dysfunction.
• Adrenocorticotropic hormone (ACTH) test. ACTH is the hormone that usually stimulates cortisol production in the adrenal glands. People with autoimmune adrenalitis can have elevated ACTH levels in their blood.
• Renin. Renin is the hormone that typically prompts production of aldosterone in the adrenal glands. Renin levels can be high in people with autoimmune adrenalitis.
• Metabolic panel. A metabolic panel can help your doctor to evaluate your blood sugar, show low sodium levels and high potassium levels in your blood, and help diagnose hypoglycemia.
• Anti-21-hydroxylase antibodies screen. Many people with autoimmune adrenalitis make autoantibodies to 21-hydroxylase that can be detected through testing.
• ACTH stimulation test. In this test, your doctor will see if levels of cortisol rise in response to an intravenous (IV) or intramuscular injection of ACTH. If little or no response is seen, it can point to problems with the adrenal glands.
• Imaging tests. Imaging technology, such as a CT scan, may be helpful in viewing your adrenal glands. It can also help determine if adrenal dysfunction may be happening due to another cause, such as an infection or cancer.

The treatment for autoimmune adrenalitis involves replacing the missing hormones. You’ll be prescribed medications that you’ll take by mouth each day for the rest of your life.

Your doctor will tailor the dose to your individual needs. In order to reduce the likelihood of side effects, ideally this will be the lowest possible dose that gets rid of your symptoms.

Cortisol may be replaced using a corticosteroid. This is typically hydrocortisone, although it’s possible that prednisone may also be used.

A medication called fludrocortisone can help with a lack of aldosterone production.

It’s also important to be aware that you’ll also need to take a higher dose of your medications when you’re experiencing added physical stress. Some examples include:

• during an illness
• before, during, and after surgery
• after experiencing an injury
• during pregnancy or delivery

You may also receive a corticosteroid injection kit. You can carry the kit with you in case an adrenal crisis comes on suddenly before help can arrive.

An adrenal crisis is treated in the hospital with IV corticosteroids and an IV saline solution that has added sugar.

There are few known risk factors for autoimmune adrenalitis. Some that we’re aware of include:

Age

Most individuals that develop autoimmune adrenalitis are between the ages of 30 and 50.

Sex

Autoimmune adrenalitis happens more commonly in people that are assigned female at birth.

Other autoimmune conditions.

It’s unknown exactly why, but having some other autoimmune diseases can put you at a higher risk of autoimmune adrenalitis. These include:

• Hashimoto’s thyroiditis
• Graves’ disease
• type 1 diabetes
• hypoparathyroidism
• hypopituitarism
• pernicious anemia
• myasthenia gravis
• vitiligo
• dermatitis herpetiformis

Remember that having risk factors for autoimmune adrenalitis doesn’t mean that you’ll definitely develop it in the future. It means that you may be at an increased risk of developing it compared with the general population.

There’s currently no way to prevent autoimmune adrenalitis.

If you’ve already received this diagnosis, in addition to taking your medications as directed, it may be helpful to add more sodium to your diet. This is because people with autoimmune adrenalitis can have lower sodium levels.

It’s important to remember to always consult your doctor or another healthcare professional before making adjustments to your diet. They can help recommend the best sources and amounts of sodium, calcium, and vitamin D for you.

One of the main complications of autoimmune adrenalitis is an adrenal crisis. This can cause:

• low blood pressure (hypotension)
• low blood sugar (hypoglycemia)
• low blood sodium (hyponatremia)
• high blood potassium (hyperkalemia)
• severe dehydration due to vomiting and diarrhea
• shock

If it’s not treated quickly, an adrenal crisis can be fatal. This condition typically comes on due to increased stress from things like:

• an injury or surgery
• an infection
• intense psychological stress or trauma

According to a 2014 review, around 50 percent of people with autoimmune adrenalitis also develop another autoimmune disease. A few examples include:

• Hashimoto’s thyroiditis
• Graves’ disease
• type 1 diabetes
• hypoparathyroidism
• pernicious anemia

When autoimmune adrenalitis is identified and treated promptly, the outlook is generally good. However, the condition does require lifelong replacement of missing hormones, which must be carefully managed by a doctor or healthcare professional.

Autoimmune adrenalitis happens when your immune system starts to attack your adrenal glands. It’s the most common cause of Addison’s disease. What exactly causes autoimmune adrenalitis is unknown.

Damage from autoimmune adrenalitis can lead to the loss of the ability to make the hormones cortisol and aldosterone. This is what causes symptoms. In times of stress, a life threatening condition called an adrenal crisis can occur.

If it’s diagnosed early, complications like an adrenal crisis can be prevented by replacing the missing hormones. Treatment for autoimmune adrenalitis is lifelong.

The initial symptoms of autoimmune adrenalitis can be vague and nonspecific. Contact your doctor for evaluation if you develop symptoms like chronic fatigue, weakness, or unexplained darkening of areas of skin.