Understanding Adrenal Insufficiency
In last month’s educational corner, we learned about the adrenal glands, their functions and the hormones they produce. In this article, we will look at what happens when the adrenal glands are not able to produce enough hormones or do not produce certain hormones at all, what this means for the body, signs to look for and management strategies.
The adrenal glands are triangular, cap-shaped glands that sit on top of our kidneys and produce important hormones for the basic functioning and maintenance of the human body. The adrenal gland consists of an outer part (cortex) and an inner part (medulla). The hormones produced vary between the cortex and medulla.
Cortex: cortisol (manages physical stress and metabolism), aldosterone (maintains blood pressure and electrolytes), and sex hormones
Medulla: adrenaline and noradrenaline (fight-or-flight hormones)
What happens when these hormones are not produced in sufficient amounts?
When there is a deficiency of hormones from the adrenal glands (especially the cortex), it can lead to a condition called adrenal insufficiency. This means that the adrenal glands are not functioning sufficiently and are not producing normal levels of hormones such as cortisol, aldosterone and adrenal androgens (sex hormones). This can affect the maintenance of basic bodily functions and how the body responds to stress, illness or infection.
With early diagnosis, intervention and prompt treatment, adrenal crisis can be prevented and most people with adrenal insufficiency can have a typical, active life.
What are the different types of adrenal insufficiency?
Primary adrenal insufficiency - This is also commonly known as Addison’s disease. It involves decreased production of aldosterone and cortisol. It can present congenitally (at birth) or occur acutely or chronically. The prevalence of primary adrenal insufficiency is estimated to be 93–140 per million.
This can occur due to many causes. One common cause is autoimmunity (70% of primary cases are due to this reason), where the body’s own immune system starts attacking the adrenal glands. This can damage the gland, resulting in lower or no production of hormones. Other less common causes of primary adrenal insufficiency include:
Internal bleeding in the adrenal gland
Genetic conditions, such as X-linked adrenoleukodystrophy (X-ALD)
Cancer in the adrenal glands
Infections such as HIV, syphilis, tuberculosis, or certain fungal infections
Exposure to certain medications
Surgical removal of the adrenal glands
For individuals with X-ALD, adrenal insufficiency is an important manifestation of the condition and can occur even in the absence of neurological symptoms.
Secondary adrenal insufficiency - This occurs when the pituitary gland produces little to no ACTH. The pituitary gland is a pea-sized gland located at the base of the brain and is considered the master gland of the body. It is responsible for producing many important hormones involved in the basic functioning and maintenance of the body.
One such hormone is ACTH, which stands for adrenocorticotropic hormone. This hormone triggers the adrenal glands to produce cortisol. When ACTH is decreased or absent, the adrenal glands produce decreased amounts of cortisol or no cortisol. This does not usually affect aldosterone levels.
Secondary adrenal insufficiency is more common than primary adrenal insufficiency, with a prevalence rate of 150–280 per million. Some of the causes of secondary adrenal insufficiency are:
Bleeding or traumatic injury to the pituitary gland or the part of the brain where the gland is located
Tumors or infections in the pituitary gland
Surgical removal of all or part of the pituitary gland
High-dose pituitary or whole-brain radiation
Genetic conditions that affect the way the pituitary gland develops or functions
Autoimmune conditions that affect the pituitary gland
Most symptoms of secondary adrenal insufficiency are similar to those of Addison's disease. However, people with secondary adrenal insufficiency do not typically develop darkened skin. They are also less likely to have serious dehydration or low blood pressure and are more likely to have low blood sugar.
Tertiary adrenal insufficiency - The most common cause of tertiary adrenal insufficiency is suddenly stopping corticosteroids after taking them for a long time. The hypothalamus is a part of the brain that helps regulate hormone production and signals the pituitary gland through a hormone called corticotropin-releasing hormone (CRH). Long-term corticosteroid use can cause higher levels of cortisol in the blood, leading the hypothalamus to make less CRH. Less CRH means less ACTH, which in turn causes the adrenal glands to reduce cortisol production. Other causes include brain tumors and surgery to treat Cushing disease (a condition in which the body is exposed to too much cortisol for a prolonged period of time).
What does adrenal insufficiency look like in terms of symptoms?
Symptoms can look different from person to person, ranging from mild to severe and, in some cases, life-threatening. They usually progress slowly over time and are often overlooked. For some individuals, however, the progression can be rapid.
The following are symptoms that may be seen in someone experiencing adrenal insufficiency. This is a collection of possible symptoms and they are not necessarily all present in one person. Sometimes, adrenal insufficiency may not show up in someone unless there is a stressor, such as an illness, infection, surgery or injury that triggers an adrenal crisis.
Extreme tiredness, also called fatigue
Dizziness or fainting when standing after sitting or lying down due to low blood pressure
Upset stomach, diarrhea, nausea or vomiting
Excessive sweating, which may occur with low blood glucose
Pain in the abdomen
Muscle cramps, weakness, widespread pain or joint pain
Loss of appetite
Weight loss
Body hair loss (which may be seen in women, particularly loss of hair in the underarms and pubic region)
Patches of darkened skin, especially on scars and moles (most commonly seen in primary adrenal insufficiency). These changes may be harder to see on skin of colour.
The dark patches may be most visible on scars, skin folds, pressure points such as the elbows, knees, knuckles, and toes, as well as the lips, and soft tissue linings such as the lining of the cheek.
Depression, anxiety, or irritable mood
Irregular or absent menstrual periods
Decreased sex drive
Salt craving
Some changes that may be seen in blood tests include:
Low blood glucose
Low sodium
High potassium, particularly in primary adrenal insufficiency
Can there be complications with adrenal insufficiency?
Yes, severe complications can occur when symptoms worsen rapidly. When medical attention is not provided in time, the body can undergo an emergency situation known as adrenal crisis (also called Addisonian crisis or acute adrenal failure). This can be fatal and requires immediate medical attention.
The body’s need for cortisol can increase during times of physical stress, such as illness, serious injury, or surgery. The body may not be able to meet these increased hormonal needs because the adrenal glands are not producing enough cortisol. The severe lack of cortisol at these times can cause life-threatening symptoms such as low blood pressure, low blood glucose, low blood sodium, and high blood potassium.
What are some emergency symptoms due to adrenal crisis?
Serious weakness.
Sudden, severe pain in the lower back, stomach area or legs.
Severe upset stomach, vomiting or diarrhea.
Extreme loss of body water, also called dehydration.
Fever.
Confusion or significantly decreased awareness of the surroundings.
Loss of consciousness.
Low blood pressure and fainting.
Call 911 or your local emergency number if you or a loved one are experiencing the above symptoms.
Treatment for adrenal crisis includes immediate intravenous (IV) hydrocortisone and large amounts of IV saline, a salt solution, with dextrose added. Dextrose is a type of sugar.
What are some precautions or important things to keep in mind?
Using a medical alert bracelet or card that provides information about the condition is important. Education about when to appropriately increase cortisol replacement doses during surgery and other stressful or infectious conditions is also vital in helping to prevent potentially life-threatening adrenal crises.
Arabic Medical Alert Bracelets for Adrenal Insufficiency
To help make important medical information more accessible, ALD Hope is providing medical alert bracelets in Arabic for individuals living with adrenal insufficiency. These bracelets can help communicate important information about a person’s condition during an emergency, particularly if they are unable to communicate for themselves.
If you or a loved one would like to request an Arabic medical alert bracelet, please complete the request form at this link: https://form.jotform.com/262684254799071
References:
Huecker, M. R., Bhutta, B. S., & Dominique, E. (2023, August 17). Adrenal insufficiency. StatPearls - NCBI Bookshelf. https://www.ncbi.nlm.nih.gov/sites/books/NBK441832/
Adrenal Insufficiency & Addison’s Disease - NIDDK. (n.d.). National Institute of Diabetes and Digestive and Kidney Diseases. https://www.niddk.nih.gov/health-information/endocrine-diseases/adrenal-insufficiency-addisons-disease
Primary adrenal insufficiency - Symptoms, diagnosis and treatment | BMJ Best Practice US. (n.d.). https://bestpractice.bmj.com/topics/en-us/56
National Library of Medicine. (n.d.). Addison Disease. Adrenal Insufficiency | MedlinePlus. https://medlineplus.gov/addisondisease.html#cat_95
Lewis, A., Thant, A. A., Aslam, A., Aung, P. P. M., & Azmi, S. (2023). Diagnosis and management of adrenal insufficiency. Clinical Medicine, 23(2), 115–118. https://doi.org/10.7861/clinmed.2023-0067
Mayo Clinic - Addison’s disease: Symptoms and causes. Mayo Clinic: Symptoms and causes
Mayo Clinic - Addison’s disease: Diagnosis and treatment. Mayo Clinic: Diagnosis and treatment
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