What are the other Names for this Condition? (Also known as/Synonyms)

• Addison Disease
• Adrenal Cortex Hypofunction
• Adrenal Cortical Insufficiency 

What is Addison’s Disease? (Definition/Background Information)

• Addison’s Disease (also known as Chronic Adrenocortical Insufficiency) is a disorder that occurs when the adrenal glands do not produce enough hormones in the body
• When any damage or dysfunction occurs to the adrenal glands, their hormone-producing ability becomes lowered and consequently, the body is unable to function properly
• Damage or dysfunction of the adrenal glands can occur in a variety of ways. The most common reason of damage is from an autoimmune reaction, where the immune system begins to make antibodies that attack cells of the adrenal cortex and destroys them
• When the damage occurs, symptoms such as fatigue, loss of appetite, and weight loss, may appear. The symptoms become more severe (called adrenal crisis), if the affected individuals are not treated promptly
• Addison’s Disease is treated with hormone therapy. With proper and lifelong treatment involving hormone replacement therapy, the prognosis is generally good

Who gets Addison’s Disease? (Age and Sex Distribution)

• Addison’s Disease affects around 1 in 100,000 individuals
• The condition is usually diagnosed in individuals by the age of 15 years. However, people of all age groups and genders may be affected
• Addison’s Disease is seen throughout the world affecting all races and ethnicities. The most common reason in the western world is autoimmune disease. In developing countries, tuberculosis is a common cause of the disorder

Exceptions to the above include:

• In one-third of the boys with Addison’s Disease, the disease is due to adrenoleukodystrophy
• Addison’s Disease due to polyglandular autoimmune (PGA) syndrome type 1 is more common in younger children
• Addison’s Disease due to PGA syndrome type 2 is 3 times more common in women

What are the Risk Factors for Addison’s Disease? (Predisposing Factors)

Following are the risk factors of Addison’s Disease:

Addison’s Disease is more likely to occur in those who are affected by other autoimmune diseases (when the body’s immune system attacks its own cells and tissues). Common autoimmune diseases associated with Addison’s Disease are:

• Chronic thyroiditis
• Graves’ disease
• Testicular dysfunction
• Hypopituitarism
• Hypoparathyroidism
• Type 1 diabetes
• Alopecia areata
• Hypogonadism
• Sjogren’s syndrome
• Vitiligo
• Pernicious anemia
• Hepatitis
• Celiac disease

Apart from the above, other risk factors for Addison’s Disease include tuberculosis and fungal infections such as coccidioidomycosis and histoplasmosis.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Addison’s Disease? (Etiology)

The causes of Addison’s Disease include:

• The two adrenal glands are situated on top of each kidney. The adrenal glands are made up of an outer cortex that produces the following hormones:

  • Glucocorticoids, which are responsible for maintaining sugar levels and immune response and helping individuals during stress
  • Mineralocorticoids, to regulate sodium and potassium levels
  • Androgens (male) and estrogens (females), which play a role in the development of sexual characteristics and sex drive
• Addison’s Disease occurs when the adrenal cortex of the adrenal gland is damaged, leading to less hormone production. The production of glucocorticoids is more commonly affected in this disorder
• The damage can occur in many ways. Autoimmune mechanisms and tuberculosis are most common causes of Addison’s Disease
• The other causes of adrenal insufficiency include cancer (lymphoma and cancers that have spread to the adrenal cortex from other organs), infections (histoplasmosis, cytomegalovirus, and syphilis), scleroderma, amyloidosis, and drugs such as mitotane
• Uncommonly, adrenocortical deficiency can also run in families. The different types seen are familial glucocorticoid deficiency,  triple A or Allgrove syndrome, and congenital adrenal hypoplasia

What are the Signs and Symptoms of Addison’s Disease?

The signs and symptoms of Addison’s Disease include:

• Fever, headache
• Loss of appetite, weight loss, fatigue
• Low blood pressure, which is worsened when the individual stands from a sitting or lying down position, lightheadedness
• Muscle weakness and muscle spasms
• Behavioral changes, irritability, anxiety, depression
• Darkening of the skin especially in pressure areas such as belt regions, nail, gums, and nipples
• Decreased body hair
• Nausea, vomiting, anorexia
• Abdominal pain, chest pain
• Joint pain, neck pain
• Problems with gait
• Problems with intellect
• Decrease in sex drive

There may be signs and symptoms of other related autoimmune diseases (or other infections) too.

How is Addison’s Disease Diagnosed?

Comprehensive evaluation of the individual’s medical history, a thorough physical examination, laboratory blood tests, and imaging tests may be conducted to diagnose Addison’s Disease.

• Blood tests are commonly done to measure the levels of sodium, potassium, cortisol, and adrenocorticotropic hormone (ACTH). If tests show increased potassium levels, low blood pressure, low cortisol and sodium levels, Addison’s Disease is a likely suspicion
• Low levels of cortisol and ACTH is common in Addison’s disease. It is further confirmed by cosyntropin stimulation test, which is done by administering ACTH and then measuring serum cortisol levels
• Complete blood count that may show decreased neutrophils and increased eosinophils and lymphocytes

Other blood tests that may be performed based on history and physical exam include very long-chain fatty acid levels, DHEA levels, 17-OH progesterone levels, epinephrine levels, and plasma renin activity.

• Since autoimmune mechanism is a common cause of Addison’s Disease, anti-adrenal antibodies may be found in half of the individuals
• Imaging studies of the adrenal and pituitary glands are also helpful in determining the cause of Addison’s Disease, if autoimmune mechanisms are not involved. Such studies may be for tuberculosis, cancer, etc.
• If other autoimmune diseases are suspected, corresponding tests to confirm or rule them out are performed

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Addison’s Disease?

Complications of Addison’s Disease include:

• Usually, hormone supplements are prescribed to treat the disorder. If too little or too much hormone supplement is taken, health complications and side effects are likely to arise
• Infection is common in individuals taking hormone replacement (cortisol) therapy
• Addison’s Disease may worsen due to associated autoimmune diseases such as Graves’ disease, hypoparathyroidism, diabetes mellitus type 1, and other conditions

When individuals do not take the hormone replacements as prescribed, or if they are stressed due to conditions such as infection, dehydration, or injury, their condition might progress into an adrenal crisis. In this condition, the symptoms get exaggerated and may lead to death, if it is not treated promptly. Thus, the affected individuals should always carry hydrocortisone injections with them.

How is Addison’s Disease Treated?

There are several treatment measures available to effectively treat Addison’s Disease. They include:

• All individuals with Addison’s Disease should be well-informed about the condition and the importance of taking hormone replacements regularly
• The affected individuals should always wear a bracelet with “Adrenal Insufficiency Takes Hydrocortisone” tag that lets other people and clinicians know about their condition, so that they could be treated accordingly
• Oral corticosteroids (tablets) such as hydrocortisone and prednisone
• Corticosteroid injections are given during an adrenal crisis
• Androgen replacement therapy may be required in some women
• Mineralocorticoids, such as fludrocortisone, are given in moderate to severe cases of the disorder
• Patients should always carry hydrocortisone injections with them for use during an emergency

Since Addison’s Disease is a chronic disorder, hormone replacement therapy must be continued throughout the patient’s life, in order to ensure that the signs and symptoms are minimal and to prevent damage to the body.

How can Addison’s Disease be Prevented?

• Currently, there are no methods to prevent the occurrence of Addison’s Disease
• Nevertheless, through hormone replacement therapy, the symptoms of the disorder can be controlled or minimized

What is the Prognosis of Addison’s Disease? (Outcomes/Resolutions)

With regular and consistent hormone replacement therapy that must be ensured throughout one’s life, most individuals with Addison’s Disease are able to lead full and normal lives.

Additional and Relevant Useful Information for Addison’s Disease:

In the United States, 4 individuals per million are diagnosed with Addison’s Disease every year.