What Is Addison’s Disease?

Addison’s disease, also known as primary adrenal insufficiency, happens when the adrenal glands don’t produce enough cortisol or aldosterone. When your body doesn’t have enough of these key hormones, you can experience symptoms like fatigue, muscle weakness, and unintended weight loss. 

Addison's disease is considered rare. It's estimated that about one out of every 100,000 people in the U.S. has Addison’s disease. Those who have Addison's disease can take hormone therapy to manage symptoms and prevent serious complications.

Addison's Disease Symptoms

Symptoms of Addison's disease usually develop slowly. They may also be similar to symptoms of other conditions, which can often delay diagnosis.

The most common symptom of Addison's disease—and among the first to develop—is fatigue. Another early symptom is hyperpigmentation. This is when darker patches of skin appear, especially near scars and skin creases and on mucous membranes like the gums.

Other symptoms of Addison’s disease can include:

• Low appetite
• Weight loss
• Dehydration
• Cravings for salty food
• Muscle weakness
• Stomach pain, nausea, and vomiting (less often, diarrhea)
• Dizziness upon standing and fainting
• Joint pain
• Mood changes, such as depression and irritability
• Low blood sugar
• Irregular periods
• Reduced libido
• Low blood pressure
• Low-grade fever

Symptoms can come and go. Symptoms are particularly more likely when you have an infection or some other type of stress on the body.

Sometimes symptoms can develop quickly. When this happens, it is known as acute adrenal failure or an addisonian crisis. Stress like surgery or a severe infection can cause this type of crisis, which can cause kidney failure and be life-threatening. Symptoms include a sudden loss of strength and severe pain in the lower back, abdomen, or legs.

What Causes Addison’s Disease?

Addison’s disease usually happens when your immune system mistakenly damages the adrenal cortex, which is the outermost layer of the adrenal glands. The adrenal glands are two hormone-producing glands on top of the kidneys. When the glands' adrenal cortex is damaged, the glands can't make enough of the hormones cortisol and aldosterone.

The two hormones are needed for many of your body's basic functions. Cortisol, also known as the stress hormone, plays a role in managing blood pressure, blood sugar, metabolism, and inflammation. Aldosterone works to manage your body’s level of sodium and potassium, which in turn helps to control your muscle and nerve function, blood pressure, and heartbeat. 

Besides the autoimmune destruction of the adrenal gland, other causes of Addison’s disease include:

• Infections like HIV, sepsis, and tuberculosis
• Certain cancers like sarcoidosis and lymphoma
• Genetic conditions that affect the adrenal glands
• Heavy bleeding into the adrenal glands
• Surgical removal of the adrenal glands as part of treatment for another condition
• Certain medications like the antifungal Nizoral (ketoconazole) or the anesthetic etomidate 

Risk Factors

Addison’s disease can affect anyone, including children. However, women and people between 30 and 50 years old are more likely to develop the condition.

You're also at greater risk for developing autoimmune-related Addison's disease if you have another autoimmune disorder, such as:

• Type 1 diabetes
• Vitiligo
• Graves' disease, a condition that can cause an overactive thyroid
• Hashimoto's disease, a condition that can cause an underactive thyroid
  

How Is Addison’s Disease Diagnosed?

Addison's disease is often first suspected after routine bloodwork shows low sodium levels or high potassium levels. Or, you might have symptoms that make your healthcare provider suspect the condition. Either way, Addison's disease can be diagnosed after a physical examination, a review of your symptoms and medical history, and a series of tests. 

The healthcare provider will measure your cortisol levels. Typically, a cortisol level greater than 18 micrograms per deciliter (mcg/dL) is normal and anything below 3 mcg/dL would be grounds for a diagnosis of adrenal insufficiency while anything below 10 mcg/dL may suggest further evaluation is needed.

To determine whether Addison's diseases is specifically causing the adrenal insufficiency, the healthcare provider will order an adrenocorticotropic hormone (ACTH) test to measure your rise of cortisol in response to stress.

Other blood tests may show:

• Low pH
• Low levels of DHEAS (dehydroepiandrosterone sulfate), a hormone that produces estrogen and testosterone
• A high eosinophil count

Your healthcare provider may also order imaging tests. For instance, a computed tomography of the abdomen can show how big the adrenal glands are.

Treatments for Addison's Disease

Addison’s disease is typically treated with steroid hormone therapy to replace the deficient hormones. 

Corticosteroids such as Cortef (hydrocortisone) and Flo-Pred (prednisolone) are taken two to three times daily to replace cortisol. Florinef (fludrocortisone) is prescribed to replace aldosterone.

Most people with Addison's disease will need to take these medications for life. Your dose may need to be adjusted during high-stress circumstances, such as:

• Pregnancy
• Injury
• Illness
• Medical emergency
• Surgery

If your Addison's disease has led to an adrenal crisis, you will need intravenous (IV) treatment of high-dose hydrocortisone and fluid, as well as electrolyte replacement.

How to Prevent Addison's Disease

There’s no way to prevent Addison’s disease. It’s typically an autoimmune disorder, which means your body’s immune system attacks its own healthy tissues. However, you can seek treatment early on if you have any symptoms to prevent complications.

Related Conditions

Having Addison's disease puts you at risk for other autoimmune conditions. In fact, about half of all people with Addison disease may develop another autoimmune condition.

Not taking the correct amount of hormone therapy can lead to other conditions. For example, taking too much glucocorticoids can lead to obesity, diabetes, and osteoporosis. A healthcare provider can help make sure you're taking the right amount.

Living With Addison's Disease

To prevent complications and manage your symptoms Addison’s disease, it’s important to take your medications regularly and exactly as prescribed. Doing this, people with Addison's disease can usually live a like-normal life. Your healthcare provider may suggest wearing a medical alert bracelet with the name of the condition on it and a note that you require steroids.

Your healthcare provider may also recommend that you make certain changes to your eating habits. If you have low aldosterone, they may suggest following a high-sodium diet. And to prevent osteoporosis from high-dose corticosteroid use, they may tell you to take in enough calcium and vitamin D or even take calcium supplements.