What is Addison’s Disease (Adrenal Insufficiency)?

What are the causes of Addison’s Disease?

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Addison’s disease is a condition that develops due to damage to the adrenal glands, resulting in insufficient production of the hormones cortisol and aldosterone in the body. The adrenal glands, which are part of the endocrine system, produce hormones that regulate the proper functioning of nearly every organ and tissue in the body.

The adrenal glands are divided into two parts: the inner portion, called the medulla, produces adrenaline-like hormones, while the outer layer, called the cortex, is responsible for the secretion of hormones called corticosteroids. Corticosteroids include the following hormones:

1. Glucocorticoids: These hormones, which contain cortisol, help convert food into energy and also assist the body in responding to situations such as stress.
2. Mineralocorticoids: These hormones, containing aldosterone, regulate the balance of sodium and potassium in the body to maintain normal blood pressure levels.
3. Androgens: Androgens are hormones secreted in the adrenal cortex of both males and females. They influence insulin sensitivity in metabolism and determine the distribution of fat and muscle tissues in the body.

Addison’s disease is classified into two groups. The first is primary adrenal insufficiency, which typically occurs due to damage to the cortex. This condition is often associated with immune system problems. Other causes of adrenal insufficiency include:

• Tuberculosis (TB)
• Other infections affecting the adrenal glands
• Spread of cancer to the adrenal glands
• Hemorrhage in the adrenal glands

Secondary adrenal insufficiency, also known as adrenocorticotropic hormone (ACTH) deficiency, occurs due to insufficient production of the hormone ACTH by the pituitary gland. Many of the symptoms seen in secondary adrenal insufficiency are similar to those seen in primary adrenal insufficiency. However, individuals with secondary adrenal insufficiency do not experience skin darkening (hyperpigmentation) and are less likely to experience severe dehydration or low blood pressure. Nevertheless, they are more likely to experience low blood sugar levels.

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What are the symptoms of Addison’s Disease?

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The symptoms of Addison’s disease typically develop slowly over several months. Often, the progression of the disease is so gradual that symptoms may not be noticeable until a stressful situation or illness occurs. Commonly observed symptoms of Addison’s disease include:

• Extreme fatigue
• Weight loss and decreased appetite
• Skin darkening (hyperpigmentation)
• Low blood pressure
• Fainting
• Increased craving for salt
• Low blood sugar (hypoglycemia)
• Nausea, diarrhea, or vomiting (gastrointestinal symptoms)
• Abdominal pain
• Muscle or joint pains
• Irritability
• Depression
• Hair loss or sexual dysfunction in women

Acute Adrenal Insufficiency (Addisonian Crisis)

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In some cases, the symptoms of Addison’s disease can manifest very quickly. This condition, known as acute adrenal insufficiency or Addisonian crisis, can be life-threatening. Therefore, it is important to seek urgent medical attention if the following symptoms are observed:

• Severe dehydration
• Cool, pale, and clammy skin
• Sweating
• Rapid breathing
• Dizziness
• Severe vomiting or diarrhea
• Weak and feeble muscles
• Headache
• Feeling of numbness or loss of consciousness

In addition to an Addisonian crisis, the following symptoms may also occur:

• Low blood pressure
• High potassium (hyperkalemia) and low sodium (hyponatremia) levels

In the hospital, a intravenous line is established in the arm to replenish fluids to address dehydration. This fluid contains a mixture of salt and sugar (sodium, glucose, and dextrose) that the body is lacking at that time. Hydrocortisone is also administered by injection to replace the deficient cortisol hormone.

When Should Medical Help Be Sought in Addison’s Disease?

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In cases where the following symptoms occur in Addison’s disease, it is crucial to seek immediate medical attention from a specialist doctor:

• Darkened areas of the skin (hyperpigmentation)
• Feelings of fatigue
• Sudden weight loss
• Stomach and intestinal problems such as nausea, vomiting, and abdominal pain
• Dizziness or fainting
• Craving for excessive amounts of salt
• Muscle and joint pains

How is Addison’s Disease Diagnosed?

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In order to diagnose Addison’s disease, the doctor first asks the patient about their medical history and symptoms. Based on this assessment, if necessary, the doctor may request some of the following tests:

• Blood tests: Blood tests measure the levels of sodium, potassium, cortisol, and adrenocorticotropic hormone (ACTH) in the blood, which are responsible for producing adrenal cortex hormones. Additionally, antibodies associated with Addison’s disease in the immune system may also be examined in blood tests.
• ACTH stimulation test: ACTH stimulates the adrenal glands to produce cortisol. This test determines the cortisol levels in the blood before and after the injection of synthetic ACTH.
• Insulin-induced hypoglycemia test: In cases where the doctor suspects adrenal insufficiency as a result of pituitary disease (secondary adrenal insufficiency), the patient may be asked to undergo this test. This test measures blood sugar and cortisol levels after insulin injection. In healthy individuals, glucose levels drop while cortisol levels rise after this test.
• Imaging tests: A computerized tomography (CT) scan of the abdomen may be used to assess the size of the adrenal glands and to identify any other abnormalities. If secondary adrenal insufficiency is detected, an MRI scan of the pituitary gland may also be necessary.

How Is Addison’s Disease Treated?

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Treatment for Addison’s disease typically involves medication to replace the deficient steroid hormones and bring their levels back to normal. Patients need to regularly take hormone-containing medications for this purpose. Some treatment options include:

1. Oral Corticosteroids: Hormones such as hydrocortisone, prednisone, or methylprednisolone are given to the patient in a manner that mimics the normal 24-hour fluctuations of cortisol levels.
2. Fludrocortisone Acetate: This medication is used to replace aldosterone.

It’s important for patients to consume a diet rich in salt, especially during periods of heavy exercise, hot weather, or gastrointestinal issues like diarrhea. Additionally, temporary increases in medication dosage under medical supervision may be necessary during times of stress on the body, such as surgery, infection, or mild illness. In cases where vomiting occurs, corticosteroid injections may be administered.

Living with Addison’s Disease

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Here are some recommendations for living with Addison’s disease:

1. Carry a Medical Alert Card and Bracelet: Patients should always carry a medical alert card and wear a bracelet. The steroid emergency card helps healthcare professionals understand the type of care the patient needs.
2. Keep Medications Accessible: Patients should keep their medications in an easily accessible place. Missing even a single day of medication can lead to serious complications. Therefore, it is important for patients to have their medications with them, even in small quantities, at work or while traveling.
3. Carry a Glucocorticoid Injection Kit: Patients should always carry a glucocorticoid injection kit with them. This kit contains a syringe and injectable corticosteroid for use in emergencies.
4. Maintain Communication with the Doctor: Patients should always stay in touch with their doctor. Continuous communication with the doctor is crucial to ensure that hormone doses are adequate and not excessive. In cases where ongoing problems with medications arise, changes may be needed in the dosage or timing of the medication. In this regard, the doctor will provide necessary information and support to the patients.
5. Attend Regular Check-ups: Regular check-ups are critical for managing the progression of this disease. Therefore, it is beneficial for patients to see their own doctor or an endocrinology specialist at least once a year without fail.