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Adrenal Insufficiency

Adrenal Insufficiency Overview

Your adrenal glands sit on top of your kidneys and produce vital hormones that help your body run smoothly. Two of the most important hormones are cortisol and aldosterone. Cortisol helps your body deal with stress, aids in controlling your blood sugar, regulates inflammation, and influences energy production. Aldosterone helps keep your blood pressure stable by managing salt and water balance.

Adrenal insufficiency is when your adrenal glands aren’t working correctly—they’re not producing enough cortisol to meet the body’s demands and, in some cases, not enough aldosterone. This can make you feel sluggish, less able to deal with physical and psychological stress, and in severe cases, lead to low blood pressure and dehydration.

Adrenal insufficiency comes in two main types:

  1. Primary Adrenal Insufficiency: This is a problem directly related to your adrenal glands, which are either damaged (autoimmune diseases, infections, and bleeding) and do not produce enough cortisol and aldosterone, or removed surgically. Autoimmune diseases are conditions where your immune system mistakenly attacks the organs in your body, such as your adrenal glands.
  2. Secondary Adrenal Insufficiency: This is when there is a problem not with the adrenal glands but with the hormones produced by the pituitary gland telling them to work. As a result, your adrenal glands do not get the message to produce sufficient amounts of cortisol to meet your body’s demands.

A lack of cortisol and aldosterone might make you feel exhausted and weak, lose weight without trying, cause low blood pressure, or even see your skin getting darker (in cases of primary adrenal insufficiency).

To determine what’s going on, your doctors can run tests to check hormone levels in your blood and see how well your adrenal glands respond to commands (with an ACTH stimulation test, for example). Treatment involves replacing these hormones with medications that act like cortisol and if needed, aldosterone, to help you feel better again.

People with adrenal insufficiency need to carry identification (like a medical alert bracelet) that lets others know about their condition in case of an emergency. Sometimes, your body needs more cortisol to deal with psychological stress or physical illness. In these circumstances, you will need to stress dose by increasing your steroid medications.

Proper management will allow you to live a healthy and active life despite having adrenal insufficiency.

About the Endocrine System

Your adrenal glands are part of your endocrine system, a highly coordinated and complex chemical messaging system within your body that is made up of specialized glands. These glands include the pituitary gland (often called the master gland), thyroid, parathyroid, adrenal glands, pancreas, and reproductive glands (ovaries in women and testes in men). These glands release chemical signals called hormones directly into the bloodstream.

Hormones are the body’s messaging molecules. They travel through the bloodstream to different body parts, delivering instructions to tissues and organs and helping regulate their function. These instructions can tell your body to grow, produce energy, respond to stress, and maintain a healthy balance of all bodily functions (homeostasis).

The master gland, the pituitary, plays a crucial role. Located at the base of your brain, it sends signals to other glands, telling them when to release their hormones. This system ensures that everything in your body works in harmony. For example, the thyroid gland controls your metabolism, the adrenal glands manage your stress response and blood pressure, and the pancreas regulates your blood sugar levels.

The endocrine system ensures messages are delivered at the right time, place, and amount. If something goes wrong within this system—for instance, if a gland produces too much or too little of a hormone—it can disrupt the balance, leading to various health issues, of which some can be serious.

Symptoms of Adrenal Insufficiency

The symptoms of adrenal insufficiency stem from your adrenal glands not producing enough cortisol and in some cases, aldosterone. These symptoms can vary in intensity, often develop gradually, and can be worsened during an illness or stressful event.

Here’s a breakdown of the symptoms you might experience with adrenal insufficiency:

  • Fatigue and Weakness: You might feel exhausted, almost like you’ve run a marathon when you haven’t. Even after a good night’s sleep, you could feel fatigued. Your muscles might feel weak, making daily tasks feel more strenuous.
  • Weight Loss and Decreased Appetite: You might notice that you’re losing weight without trying. Along with this, your appetite might decrease, and you could find yourself eating less than usual.
  • Low Blood Pressure: Your blood pressure may drop, making you feel dizzy or lightheaded, especially when you stand up from sitting or lying down. This happens because there’s insufficient aldosterone to keep your blood pressure normal.
  • Salt Craving: Due to the low aldosterone levels, your body may lose more salt than it should, leading to cravings for salty foods.
  • Low Blood Sugar: Since cortisol helps manage your blood sugar levels, not having enough can lead to hypoglycemia, especially in children. You might feel more irritable or shaky than usual.
  • Nausea, Diarrhea, or Vomiting: Digestive issues are common, and you might find yourself feeling nauseous and experiencing bouts of diarrhea or vomiting.
  • Skin Changes: In primary but not secondary adrenal insufficiency, areas of your skin and mucous membranes (like the inside of your mouth) may turn darker or more pigmented. This symptom is more specific to Addison’s disease, a type of primary adrenal insufficiency.
  • Mood Changes: You might experience mood swings, depression, or irritability. The lack of cortisol can affect your emotional well-being.

These symptoms can initially be subtle and are often mistaken for signs of other illnesses. If you’re experiencing these symptoms, it’s essential to consult a healthcare provider who can perform tests to diagnose adrenal insufficiency. An accurate diagnosis will help you to manage the condition effectively, minimizing these symptoms and improving your quality of life.

Treatments for Adrenal Insufficiency

Treating adrenal insufficiency focuses on replacing the hormones your adrenal glands cannot produce sufficiently. Treatment aims to mimic the expected levels of these hormones to help you feel better and function normally.

The critical components of treatment include:

  • Steroid Replacement: This is the cornerstone of treatment. Medications such as hydrocortisone, prednisone, or dexamethasone are used to replace cortisol, the hormone that your body is lacking. The specific medication and dose depend on your individual needs. Your doctor will adjust them to mimic the natural rhythm and amount of cortisol your body would typically produce.
  • Mineralocorticoid Replacement: If you have primary adrenal insufficiency, your body may also lack aldosterone, another vital hormone the adrenal glands produce. In this case, you would be prescribed a medication called fludrocortisone to help maintain your body’s balance of salt and water, which is crucial for controlling blood pressure.
  • Androgen Replacement: Some, but not all, women with adrenal insufficiency may benefit from androgen replacement therapy. DHEA (dehydroepiandrosterone) is a weak male hormone (androgen) produced by the adrenal glands. Supplementing with DHEA can help improve overall well-being, energy levels, and sex drive in some women.
  • Adjusting Medication During Stress: It’s crucial to increase your steroid dosage during periods of stress, such as illness, surgery, or even severe emotional stress. Since your body would increase cortisol production in these situations, you must mimic this response by taking more steroids. Your doctor will guide you on adjusting your doses in these circumstances.
  • Emergency Treatment: If you have adrenal insufficiency, you’re at risk of adrenal crisis, a life-threatening situation that requires immediate medical attention. Symptoms include severe vomiting, diarrhea, low blood pressure, and loss of consciousness. You should always carry a medical alert card or wear a medical alert bracelet that lets others know of your condition in an emergency. Injectable hydrocortisone should be kept on hand for such situations, and you and your loved ones should know how and when to use it. For more on adrenal crisis, please see below.
  • Regular Monitoring: Regular follow-ups with your healthcare provider are essential. These appointments are crucial for monitoring your condition, adjusting medication as needed, and ensuring that you’re receiving the optimal treatment.

These treatments aim to keep your symptoms under control and prevent complications, enabling you to lead a healthy and active life. Your healthcare team will work closely with you to tailor your treatment plan to your needs and lifestyle.

Adrenal Crisis

Adrenal crisis is a serious and potentially life-threatening condition that occurs when your body is under severe stress, and your adrenal glands fail to produce enough cortisol to meet its demands. It’s most commonly seen in individuals with adrenal insufficiency, whose adrenal glands don’t make enough hormones under normal circumstances. The demand for cortisol spikes dramatically during periods of acute stress, such as illness, surgery, or severe injury. The body’s inability to meet this demand can lead to adrenal crisis.

The symptoms of an adrenal crisis can develop quickly and may include:

  • Severe weakness
  • Fatigue and confusion
  • Abdominal pain (sometimes severe), vomiting, and diarrhea leading to dehydration
  • Low blood pressure, causing dizziness or fainting
  • Low blood sugar levels
  • Fever
  • Rapid heart rate

If you or someone you know with adrenal insufficiency begins to experience these symptoms, it’s crucial to seek emergency medical attention immediately. Adrenal crisis is a medical emergency that requires prompt treatment.

The primary treatment for an adrenal crisis involves the immediate administration of intravenous (IV) or intramuscular (IM) hydrocortisone, a steroid that acts like cortisol. IV fluids often accompany this to combat dehydration and restore normal blood pressure and blood sugar levels. Once stabilized, doctors will continue to monitor and adjust your treatment as necessary.

For individuals with adrenal insufficiency, preventing an adrenal crisis is critical. Taking the following steps can decrease your risk of experiencing adrenal crisis:

  • Medication Adherence: Always take your prescribed medications as directed to maintain sufficient cortisol levels.
  • Stress Doses: Learn how to adjust your medication during stress or illness under the guidance of your healthcare provider. This often means increasing your dose of steroids.
  • Emergency Injectable Hydrocortisone: Keep an emergency kit containing injectable hydrocortisone, and make sure you and those close to you know how to use it if you cannot take your oral medications.
  • Medical Alert Identification: Wear a medical alert bracelet or carry a card that indicates you have adrenal insufficiency, which can inform healthcare professionals of your condition in an emergency.

Understanding the signs of an adrenal crisis and knowing the steps to take in an emergency can effectively manage this condition and reduce your risk. Regular follow-ups with your healthcare provider are also essential to ensure your treatment plan remains appropriate for your needs.

Common Questions about Adrenal Insufficiency

How common is adrenal insufficiency?

Adrenal insufficiency is relatively rare compared to other diseases like diabetes and high blood pressure, and its exact prevalence can vary depending on the type and the geographic location.

Primary Adrenal Insufficiency

Addison’s disease, the most common form of primary adrenal insufficiency, has an estimated prevalence of about 110 to 144 cases per million people in developed countries.

The incidence rate, or the rate of new cases, is roughly four-to-six new cases per million people per year.

Addison’s disease can occur at any age, in any gender, and in all racial and ethnic groups. However, it most commonly presents in adults.

Secondary Adrenal Insufficiency

Secondary adrenal insufficiency is more common than primary adrenal insufficiency. However, specific prevalence rates can be challenging to determine due to the variety of underlying causes, such as pituitary tumors or the prolonged use of steroid medications that can suppress the body’s natural cortisol production.

It’s estimated that the prevalence might be higher, given that steroids are used very commonly in a wide range of conditions, and is a significant risk factor for developing secondary adrenal insufficiency.

Factors Affecting Prevalence

The prevalence of adrenal insufficiency can be influenced by several factors, including the criteria used for diagnosis, the awareness of the condition among healthcare providers, and regional differences in autoimmune diseases and genetic factors.

It is crucial for adrenal insufficiency to be recognized and treated promptly due to the serious risks associated with adrenal crisis, a life-threatening condition that can occur if the disease is missed or not adequately managed. Awareness and education about the signs and symptoms of adrenal insufficiency can help improve diagnosis rates and outcomes for those affected by this condition.

Does adrenal insufficiency cause weight gain?

Adrenal insufficiency typically does not cause weight gain; it is more commonly associated with weight loss. One of the hallmark symptoms of adrenal insufficiency is unexplained weight loss, along with other symptoms like fatigue, muscle weakness, low blood pressure, and craving for salt.

However, the treatment for adrenal insufficiency often involves steroid replacement therapy to substitute for the deficient cortisol. Steroids, such as hydrocortisone, prednisone, or dexamethasone, mimic the effects of cortisol. While these medications are critical for managing adrenal insufficiency, long-term overt-treatment of high doses of steroids can cause side effects that include weight gain, increased appetite, and the redistribution of fat to the face, neck, and abdomen.

It’s essential for people receiving steroid therapy to be monitored closely by their healthcare provider. Using the lowest effective dose of steroids to alleviate the symptoms of adrenal insufficiency while minimizing the risk of side effects, including weight gain, is the goal. If you’re concerned about weight changes or other side effects from your medication, you should discuss these concerns with your healthcare provider. They can adjust your treatment plan to manage your condition and reduce the risk of side effects.

Do other diseases or conditions mimic adrenal insufficiency?

Several conditions can mimic adrenal insufficiency due to the overlap in symptoms, making diagnosis challenging. Adrenal insufficiency presents with nonspecific symptoms such as fatigue, weakness, weight loss, and abdominal pain, which are common to many other diseases. Some conditions that can cause similar symptoms include:

  • Thyroid Disorders: Both hypothyroidism (underactive thyroid) and hyperthyroidism (overactive thyroid) can mimic symptoms of adrenal insufficiency, such as fatigue, weight changes, and mood disturbances.
  • Chronic Fatigue Syndrome: Characterized by extreme, unexplained fatigue that doesn’t improve with rest and worsens with physical or mental activity, which can be confused with the fatigue seen in adrenal insufficiency.
  • Fibromyalgia: A condition marked by widespread musculoskeletal pain accompanied by fatigue, sleep, memory, and mood issues, can mimic the muscle pain and fatigue of adrenal insufficiency.
  • Autoimmune Diseases: Conditions like systemic lupus erythematosus (SLE) or rheumatoid arthritis (RA) can have overlapping symptoms with adrenal insufficiency, including fatigue, joint pain, and muscle weakness.
  • Infections: Chronic infections, such as HIV/AIDS and tuberculosis, and COVID-19 can lead to symptoms similar to those of adrenal insufficiency.
  • Depression and Anxiety: These mental health conditions can cause fatigue, appetite changes, and weight changes, similar to adrenal insufficiency.
  • Addison’s Disease: Though Addison’s disease is a form of primary adrenal insufficiency, its early symptoms can mimic those of secondary adrenal insufficiency, making it essential to differentiate between the two through diagnostic testing.
  • Hypopituitarism: A condition where the pituitary gland doesn’t produce enough of one or more of its hormones, which can lead to symptoms similar to secondary adrenal insufficiency because the pituitary gland produces ACTH (adrenocorticotropic hormone), which stimulates the adrenal glands.
  • Eating Disorders: Anorexia nervosa and other eating disorders can result in weight loss and fatigue, similar to adrenal insufficiency.

Because of this symptom overlap, healthcare providers must conduct thorough medical histories, physical examinations, and specific diagnostic tests to differentiate adrenal insufficiency from these other conditions. This might include hormone level tests, ACTH stimulation tests, imaging studies, and other assessments tailored to the suspected underlying condition.

What is the difference between adrenal insufficiency and Addison’s disease?

Addison’s disease and adrenal insufficiency are closely related but not the same.

Adrenal insufficiency is a broader term that describes a condition where the adrenal glands do not produce adequate amounts of certain hormones, primarily cortisol and, in some cases, aldosterone. Adrenal insufficiency can be classified into two main types based on its origin:

Primary adrenal insufficiency occurs when the problem lies directly within the adrenal glands. They fail to produce enough hormones due to damage or disease affecting the glands. Addison’s disease is a specific cause of primary adrenal insufficiency.

Secondary Adrenal Insufficiency occurs when there is a problem with the pituitary gland resulting in decreased production of ACTH. ACTH is the hormone that stimulates the adrenal glands to produce cortisol. If the pituitary gland doesn’t produce enough ACTH, the adrenal glands won’t produce enough cortisol. Secondary adrenal insufficiency does not typically affect aldosterone production.

Addison’s Disease

Addison’s disease is a specific type of primary adrenal insufficiency. In this condition, the body’s immune system mistakenly attacks the adrenal glands, damaging them and impairing their ability to produce hormones is the most common cause. Other causes include infections, bleeding into the adrenal glands, and genetic factors.

The difference between Addison’s disease and other forms of adrenal insufficiency is that Addison’s specifically refers to adrenal damage due to autoimmune disease or other direct damage to the adrenal glands, leading to a deficiency in cortisol and often aldosterone. In contrast, adrenal insufficiency includes any condition, primary or secondary, that results in inadequate production of adrenal hormones.

Videos

Resources

NIH: Definition & Facts of Adrenal Insufficiency & Addison’s Disease

Medline Plus: Addison Disease

Group 49
  Cases per Million People

Primary adrenal insufficiency has an estimated prevalence of about 110 to 144 cases per million people in developed countries

References

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Medically Reviewed by Kevin C.J. Yuen, MBChB, MD, FRCP, FACE on March 12, 2024