What is Addison’s Disease?
Addison’s disease (also called chronic adrenal insufficiency, hypocortisolism, and hypoadrenalism) is a very rare endocrine disorder that occurs when the adrenal glands are damaged and do not function properly. The damaged adrenal glands produce insufficient amounts of the hormones cortisol and aldosterone, which results in a number of non-specific symptoms such as pain and weakness. Under certain conditions, though, the symptoms of Addison’s Disease may progress to Addisonian Crisis, which is a severe condition that can include extremely low blood pressure, death, and coma.
Addison’s Disease is named after Thomas Addison, who first described the condition.
Addison’s disease occurs throughout all age groups and both sexes, and can be life-threatening if not properly treated. Treatment of Addison’s disease involves taking replacement hormones to replace the insufficient amounts produced by the damaged adrenal glands, which behaves to mimic the beneficial effects from the natural hormones.
- Primary adrenal failure may be caused by damage by the immune system. Certain infections can also cause adrenal crisis.
- Secondary adrenal failure occurs when there is a reduction in the production of corticotropin, which is produced by the pituitary gland. Corticotropin causes stimulation of the adrenal gland. Secondary adrenal failure can also occur when corticosteroid use is abruptly discontinued in those with another chronic illness.
- Tertiary adrenal failure occurs when the hypothalamus does not produce enough hormone to stimulate the pituitary gland to release the corticotropin
What Causes Addison’s Disease?
Addison’s disease is caused by damage to the adrenal glands, leading to insufficient amounts of the hormone cortisol and aldosterone. The adrenal glands are located just above the kidney, and are a part of the body’s endocrine system, they produce hormones that control virtually every organ and tissue in the body.
The adrenal glands of the body are made of two sections:
- Medulla – the interior portion of the adrenal gland that produces adrenaline-like hormones.
- Cortex – the outer layer produces a group of hormones called “corticosteroids,” which include androgens (male sex hormones), glucocorticoids, and mineralcorticoids.
These hormones are essential for survival. Let’s explore the role of these hormones:
- Androgens: these are the male sex hormones that are produced in small amounts by the adrenal glands in men AND women. Androgens are responsible for sexual development in men and women, and influence libido, muscle mass, and a sense of well-being for both sexes.
- Glucocorticoids – these hormones, which include cortisol, influence the ability of the body to convert food into energy as well as aid in the inflammatory response of the immune system, and help the body respond to stress.
- Mineralocorticoids – these hormones, which include aldosterone, maintain sodium and potassium balance within the body to ensure normal blood pressure.
Addison’s disease develops when the cortex of the adrenal glands are damaged and don’t produce sufficient amounts of the hormones.
Any condition that leads to damage of the adrenal glands is called “Primary Adrenal Insufficiency.”
Possible causes of damage to the adrenal glands can include:
An autoimmune condition that causes the immune system to mistakenly attack the adrenal gland is the primary cause for Addison’s Disease. Other causes for adrenal failure can include:
- Certain infections like HIV, fungal infections, and tuberculosis.
- Other infections of the adrenal glands
- Cancer that’s metastasized into the adrenal glands
- Bleeding into the adrenal glands (from hemorrhage or use of blood-thinning agents)
Secondary Adrenal Insufficiency occurs if the pituitary gland is diseased. The pituitary gland creates a hormone called adrenocorticotropic hormone (ACTH), which stimulates the adrenal cortex to produce hormones. Improper production of ACTH can lead to insufficient production of the hormones created by the adrenal glands, although it’s worth noting that the adrenal glands are not damaged.
Secondary Adrenal Insufficiency can occur among those who’ve used corticosteroids to treat chronic conditions such as arthritis or asthma, and then abruptly stop treatment with corticosteroids.
Addisonian Crisis if Addison’s disease remains untreated, an Addisonian Crisis can be provoked by physical stress, like an injury, infection, or illness.
Risk Factors for Addison’s Disease:
While there are no certain risk factors that lead to the development of Addison’s Disease, there are a number of additional autoimmune diseases that increase the risk for the autoimmune-type of Addison’s disease. These risk factors include:
- Cancer
- Chronic thyroiditis
- Dermatitis herpatoformis
- Graves’ disease
- Hypoparathyroidism
- Hypopituitarism
- Myasthenia gravis
- Pernicious anemia
- Testicular Dysfunction
- Type 1 diabetes
- Vitiligo
What Are The Symptoms of Addison’s Disease?
The symptoms of Addison’s Disease are often non-specific and typically develop over the course of several months. Symptoms of Addison’s Disease can include:
- Chronic diarrhea
- Hyperpigmentation (darkening of the skin)
- Extreme hypotension, possibly fainting.
- Extreme muscle weakness
- Changes in blood pressure or heart rate
- Hypoglycemia
- Muscle and/or joint pain
- Fatigue
- Craving salt
- Slow or sluggish movement
- Depression
- Nausea/vomiting
- Loss of libido
- Body hair loss
- Unintentional weight loss
Acute Adrenal Failure (Addisonian Crisis) Symptoms:
For some people, the signs of Addison’s Disease may appear very suddenly. Symptoms of Addisonian Crisis can include the following:
- Severe, extreme vomiting and diarrhea
- Dehydration
- Hypotension
- Loss of consciousness
- Pain in the lower back, legs or abdomen
- Hyperkalcemia
How Is Addison’s Disease Diagnosed?
A physician will diagnose Addison’s disease with the help of blood testing and imaging after taking an inventory of presenting symptoms.
Laboratory Blood Tests are able to measure cortisol, sodium, potassium and ACTH levels in the blood. This will indicate whether or not an adrenal insufficiency may be present. Antibodies will also be measured to ascertain if autoimmune Addison’s disease is present.
ACTH Stimulation Test – this test measures the level of cortisol in the blood before and after an injection of synthetic ACTH. ACTH works by stimulating the adrenal glands to produce cortisol. If there is damage to the adrenal glands (primary adrenal insufficiency), the response to ACTH in the blood will show reduced or absent amounts of cortisol.
Insulin-Induced Hypoglycemia Test – if the doctors suspect secondary adrenal insufficiency, this test may be performed. It works by checking the blood glucose and cortisol levels at various intervals after an injection of insulin. In healthy people, blood glucose levels fall and cortisol levels rise.
Medical Imaging Scans – these imaging studies, which can include an ultrasound and/or an MRI can be used to ascertain the size of the adrenal glands as well as check for any abnormalities that may give reason for the adrenal insufficiency. An MRI of the pituitary gland may be performed to ascertain whether or not Addison’s disease is caused by secondary adrenal insufficiency.
What Is Treatment For Addison’s Disease?
Treatment for Addison’s disease involves replacement corticosteroids and hormone replacement. This treatment will control the symptoms of the disease and most people receiving this treatment are able to lead normal lives. Some options for treatment may include the following:
Androgen Replacement Therapy – Many women with insufficient androgen levels find that taking androgen replacements can do wonders for their well-being, libido, and sexual satisfaction.
Corticosteroid Injections – if oral medications aren’t tolerated, corticosteroid injections may be necessary instead.
Oral Corticosteroids – oral steroid treatment may be used to manage Addison’s Disease.
Also included in treatment of Addison’s Disease is the ingestion of large amounts of sodium, especially during hot weather or if diarrhea is present. Dosages may increase if there is an illness, operation, infection, or other stressful situations.
Addison’s disease requires life-long treatment, including regular medical care and monitoring. During times of injury, infection, or stress, additional dosage of corticosteroids may be necessary. It is recommended that those with Addison’s disease wear a medical alert bracelet in case of emergency.
Addisonian Crisis: an Addisonian crisis is a life-threatening situation leading to hypotension, hypoglycemia, and high levels of potassium. This is a medical emergency. Treatment for an Addisonian Crisis includes:
- Dextrose
- Hydrocortisone
- Saline solution
What Are Some Complications of Addison’s Disease?
Some complications are possible if too much or too little medication is administered. Problems that can occur with Addison’s Disease are:
Illness – during illness, oral dosages of medication may be adjusted to mimic the normal response of the adrenal glands to the stress on the body. Significant fever or injury may require triple dosages. When a person with Addison’s recovers, the dosage will be returned to normal.
Pregnancy – women with adrenal insufficiency who become pregnant, should be treated with the standard hormone replacement therapy. If the first trimester involves nausea and vomiting, she should switch to injections of the hormones.
Surgery – as cortisol is a stress hormone, those with Addison’s Disease who need surgery under a general anesthesia need to be treated with IV glucocorticords and saline until the person is able to eat and drink. The dosage will be adjusted until the person recovers from surgery.
What is An Adrenal or Addisonian Crisis?
If Addison’s disease is untreated or not properly controlled, an adrenal crisis can occur if the individual is physically injured, ill, or fighting an infection. Symptoms of adrenal crisis include reduced consciousness, difficulty breathing, abdominal pain, and/or low blood pressure. Warning signs include:
- Unable to take medication due to vomiting
- Stress, injury, trauma, infection, or dehydration
- Weight gain
- Swollen ankles
Living With Addison’s Disease:
Addison’s Disease is a chronic condition that requires medical management and adherence to all medication prescribed. Coping with a chronic illness can feel overwhelming at times. Here are some tips for living with Addison’s Disease:
Find a local support group and join it. Finding people who are going through what you are going through can be immensely comforting.
Write about your feelings and your fears, either in a journal, or here on Band Back Together.
Stay in contact with your doctor. An ongoing relationship with a doctor you trust is vital to management of Addison’s Disease.
Keep extra medications on hand as missing even one dose can be dangerous to your health.
Wear a medical bracelet. That way, if you’re incapacitated, EMT’s will know how to care for you.
Additional Addison’s Disease Resources:
National Adrenal Diseases Foundation: non-profit organization dedicated to providing support, information and education to individuals having Addison’s disease as well as other diseases of the adrenal glands.
Pituitary Network Association: international non-profit organization for patients with pituitary tumors and disorders, their families, loved ones, and the physicians and health care providers who treat them.
The Addison’s Society of Canada: Offers Canadian support and information to people who have been diagnosed with Addison’s Disease
Last audit 7/2018