What is Adrenal Insufficiency?
Addison’s disease is a clinical condition characterized by insufficient cortisol secretion from the adrenal cortex. Pathophysiologically, a glucocorticoid (cortisone) deficiency that occurs within the scope of an adrenal gland disease is called primary adrenal insufficiency. The form caused by a deficiency of ACTH (Adrenocorticotropic Hormone) is called secondary adrenal insufficiency. Primary adrenal insufficiency was first described by Thomas Addison in 1855. While tuberculosis was the most common cause at the time of Addison’s description, today autoimmunity is the most frequent cause of the disease.
The most common cause of secondary adrenal insufficiency is the long-term use of exogenous glucocorticoids (cortisone preparations) in supraphysiological doses. This leads to chronic suppression of the Hypothalamic-Pituitary-Adrenal Axis (HPA axis). If these medications are abruptly discontinued, patients remain susceptible to steroid deficiency. The recovery of adrenal function significantly depends on the total dose of the previously administered glucocorticoid and the duration of use.
What Is Addison's Disease?
Addison’s disease, also known in medical literature as primary adrenal insufficiency, is a rare health disorder that occurs when the adrenal glands do not produce enough vital hormones the body needs. The walnut-sized adrenal glands, located directly above the kidneys, are particularly responsible for producing the hormones cortisol and aldosterone. In Addison’s disease, however, these glands produce these hormones below the required levels.
Causes Of Addison's Disease
The cause of the disease is divided into two main groups, depending on whether the damage is to the glands themselves or to the center that controls them:
- Primary Adrenal Insufficiency (Addison’s Disease) In this case, the outer layer (cortex) of the adrenal glands is directly damaged. This damage leads to insufficient production of vital hormones such as cortisol and aldosterone.
- Autoimmune Attack: The most common cause is the body’s own immune system mistakenly attacking the adrenal glands. Individuals with Addison’s disease have a higher risk for other autoimmune diseases.
- Infections: Severe infections like tuberculosis or other adrenal infections can permanently damage the glands.
- Cancer Spread: In rare cases, the spread of cancer from another organ to the adrenal glands.
- Adrenal Hemorrhage: Sudden and severe bleeding in the glands can lead to an Addisonian crisis without prior symptoms.
- Genetic Factors: Congenital structural or functional disorders of the glands, such as congenital adrenal hyperplasia (CAH).
- Certain Medications: Specific medications that suppress the body’s ability to produce cortisol or inhibit its effect.
- Effects Of Cancer Therapies: Certain cancer therapies (e.g., checkpoint inhibitors) can rarely cause adrenal insufficiency.
- Secondary Adrenal Insufficiency In this case, the adrenal glands are healthy, but the problem lies in the pituitary gland in the brain, which should stimulate them. The pituitary gland produces insufficient ACTH (Adrenocorticotropic Hormone), which stimulates the adrenal glands to produce hormones. Without sufficient ACTH stimulation, the glands cannot produce enough hormones.
The main causes of this pituitary ACTH production disorder are:
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- Pituitary Problems: Benign tumors in the pituitary gland, surgical interventions in this area, radiation therapy, or head injuries.
- Abrupt Discontinuation Of Corticosteroid Medications: Patients who have taken corticosteroids for chronic diseases like asthma or rheumatism for a long time and discontinue them abruptly can develop temporary insufficiency. These medications should be reduced under medical supervision and gradually.
In secondary adrenal insufficiency, skin darkening usually does not occur, and the risk of severe fluid loss or low blood pressure is lower. However, low blood sugar levels (hypoglycemia) can occur more frequently.
Symptoms Of Addison's Disease
The symptoms of Addison’s disease typically have a gradual onset and develop slowly over months. Often, the disease progresses so slowly that affected individuals ignore the initial symptoms. However, physical stress such as illness or injury can lead to a rapid worsening of symptoms.
Specialist Uzm. Dr. Burcu Meryem Atak Sançmış lists the early symptoms of Addison’s disease as follows:
- Extreme Fatigue (Exhaustion): A persistent feeling of intense tiredness that does not disappear with rest.
- Orthostatic Hypotension: Low blood pressure, characterized by dizziness or fainting when standing up from a sitting or lying position.
- Hypoglycemia (Low Blood Sugar): Low blood sugar levels, which can lead to sweating.
- Gastrointestinal Complaints: Digestive problems such as nausea, vomiting, diarrhea, and abdominal pain.
- Muscle And Joint Pain: Muscle cramps, general muscle weakness, or joint pain.
- Loss Of Appetite And Unintentional Weight Loss: Decreased appetite and associated unexplained weight loss.
- Skin Changes (Hyperpigmentation): Darkening of the skin, especially on scars, moles, joint creases, lips, and nail beds. This can be more difficult to detect in individuals with darker skin.
- Reduction Of Body Hair: Especially in women, body hair loss.
- Emotional And Psychological Changes: Symptoms such as depression, irritability, low sexual desire (libido).
Intense Craving For Salt: A strong craving for salty foods due to a disturbance in the body’s salt balance.
Symptoms Of Acute Adrenal Insufficiency (Addisonian Crisis)
In some cases, the symptoms of Addison’s disease can appear suddenly and severely. This condition is called acute adrenal insufficiency or Addisonian crisis and is a life-threatening emergency. This crisis is characterized by one of the following symptoms and requires immediate medical attention:
- Severe Weakness: Sudden and extreme loss of strength.
- Sudden, Terrible Pain: Suddenly occurring severe pain in the lower back, abdominal area, or legs.
- Severe Gastrointestinal Symptoms: Severe nausea, vomiting, and diarrhea, often leading to dehydration.
- Changes In Mental State: Confusion, delirium, disorientation, or loss of consciousness.
- Fever.
- Low Blood Pressure And Fainting.
- Electrolyte Imbalances: High potassium levels (hyperkalemia) and low sodium levels (hyponatremia).
Diagnosis Of Addison's Disease
The diagnosis of Addison’s disease can sometimes be time-consuming due to the variety of symptoms and its slow progression. Specialist Uzm. Dr. Burcu Meryem Atak Sançmış uses a careful clinical assessment in combination with various laboratory tests for an accurate diagnosis.
The diagnostic process usually includes the following steps:
- Medical History And Physical Examination: Your doctor will first take a detailed medical history, asking about your symptoms and their duration. This is followed by a physical examination to assess your general health. Symptoms such as skin darkening (hyperpigmentation), blood pressure changes, and general weakness are carefully examined.
- Blood Tests: Blood tests play a key role in the diagnosis of Addison’s disease. These tests include:
- Cortisol Levels: This is measured in the early morning when cortisol levels are highest. Low cortisol levels can indicate Addison’s disease.
- ACTH (Adrenocorticotropic Hormone) Levels: When cortisol production is low, the pituitary gland produces more ACTH to stimulate the adrenal glands. In primary adrenal insufficiency, ACTH levels are usually elevated.
- Aldosterone And Renin Levels: Aldosterone levels may be low and renin levels high. This indicates a disturbance in the system that regulates the body’s salt and water balance.
- Sodium And Potassium Levels: Low sodium levels (hyponatremia) and high potassium levels (hyperkalemia) in the blood are common findings in Addison’s disease.
- Autoantibodies: If autoimmune Addison’s disease is suspected, the presence of antibodies against the adrenal cortex (especially 21-hydroxylase antibodies) is investigated. The detection of these antibodies confirms autoimmune Addison’s disease.
- ACTH Stimulation Test: This test is used to assess how the adrenal glands respond to ACTH. Blood cortisol levels are measured before and after the injection of synthetic ACTH. Normally, ACTH injection leads to a significant increase in cortisol levels. However, in individuals with Addison’s disease, cortisol levels do not rise because the adrenal glands are damaged. Specialist Uzm. Dr. Burcu Meryem Atak Sançmış points out that if secondary adrenal insufficiency is suspected, alternative tests such as the low-dose ACTH stimulation test or the long-term ACTH stimulation test may also be performed.
- Imaging Techniques:
- Abdominal Tomography (CT): Can be performed to check the size of the adrenal glands and look for abnormalities (such as infections, bleeding, tumors).
- Pituitary MRI: If secondary adrenal insufficiency is diagnosed, an MRI scan may be necessary to investigate problems (such as tumors) in the pituitary gland.
Treatment Of Addison's Disease
The basic treatment methods are:
- Hormone Replacement Therapy (Medical Treatment):
- Glucocorticoids: Medications such as hydrocortisone, prednisone, or methylprednisolone are taken orally to replace the cortisol that the body does not produce sufficiently. These medications are adjusted according to a schedule that mimics the natural 24-hour fluctuations of cortisol levels in the body. Specialist Uzm. Dr. Burcu Meryem Atak Sançmış points out that the dosage can be adjusted to the patient’s daily activity level and stress factors.
- Mineralocorticoids: Fludrocortisone acetate is administered to address aldosterone deficiency and maintain the body’s sodium-potassium balance. Especially in hot weather, during intense physical activity, or with digestive problems such as diarrhea, additional salt intake (sodium) through the daily diet may be required upon medical advice.
- Dose Adjustments In Special Situations: In times of physical stress such as surgery, infections, severe illnesses, or trauma, the body’s cortisol requirements increase beyond normal levels. In these situations, Specialist Uzm. Dr. Burcu Meryem Atak Sançmış may recommend a temporary increase in medication dosage. For patients who cannot take oral medications due to vomiting or similar reasons, corticosteroid injections may be necessary.
- Treatment Of Addisonian Crisis: Addisonian crisis is a medical emergency characterized by symptoms such as low blood pressure, low blood sugar, and high potassium levels. This crisis is typically treated with high-dose corticosteroids and fluid replacement intravenously.
- Lifestyle Changes And Home Care: It is important for people with Addison’s disease to take certain precautions to improve their quality of life and prevent an Addisonian crisis:
- Medical Emergency ID: Constantly carrying a medical emergency ID indicating Addison’s disease is crucial in emergencies so that medical personnel can take the correct intervention.
- Emergency Plan: You should create a written emergency plan with your doctor that describes what to do in case of a crisis and when symptoms worsen.
- Keep Spare Medication Handy: Since it can be dangerous to interrupt treatment even for one day, it is crucial to keep spare medication at work and while traveling.
- Glucocorticoid Injection Kit: Upon medical advice, it is important to carry a kit with injectable corticosteroids for emergencies.
- Regular Check-ups: At least once a year, you should visit Specialist Uzm. Dr. Burcu Meryem Atak Sançmış or an endocrinologist to regularly monitor hormone levels and screen for possible other autoimmune diseases.
Frequently Asked Questions
Is Addison's Disease Hereditary?
Addison’s disease is usually the result of a hereditary predisposition. Most often, it is an autoimmune disease where the immune system attacks its own adrenal glands. This means that genetic factors can play an important role in the development of the disease.
What Is An Addisonian Crisis And What Is To Be Done?
An Addisonian crisis is a life-threatening emergency characterized by symptoms such as a drop in blood pressure, a drop in blood sugar, and severe weakness. In this case, you must immediately call emergency services (e.g., 112 in Türkiye, or your local emergency number) and seek medical help.
Is Addison's Disease Curable?
There is no definitive cure for Addison’s disease, but it can be successfully managed with lifelong hormone replacement therapy, which replaces the missing hormones.
Do People With Addison's Disease See Side Effects From Corticosteroids?
The corticosteroid doses used in treatment cover the body’s missing hormone needs, so the severe side effects of high-dose corticosteroids used in the treatment of other diseases generally do not occur in Addison’s patients. However, if weight gain is noted, cortisone medications and dose adjustments should be made as part of the treatment through regular doctor visits.
Is Addison's Disease Preventable?
No, Addison’s disease is not a preventable disease. However, regular treatment and adherence to medical recommendations are crucial to prevent an Addisonian crisis.
Can A Woman With Addison's Disease Become Pregnant?
With proper treatment and medical supervision, women with Addison’s disease can become pregnant. However, medication changes and dose adjustments may be necessary during pregnancy.
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