A rare hormonal or endocrine disorder which affects around 1 in 100,000 people, Addison’s disease happens in any age group and afflicts women and men equally. Symptoms from this disease include muscle weakness, weight loss, low blood pressure, fatigue and occasionally darkening of the skin in both non-exposed and exposed parts of the body.

Cortisol and Addison’s Disease

When the adrenal glands don’t make enough of the hormone cortisol and in some cases, aldosterone as well, Addison’s disease occurs. These low cortisol levels are the reason the disease is sometimes known as chronic adrenal insufficiency, or hypocrotiosolism.

Normally made by the adrenal glands which are found right above the kidneys, cortisol belongs to a class of hormones known as glucocorticoids. These affect just about every tissue and organ in the body. The most important job of cortisol is to assist the body in response to stress. Besides its other vital tasks, cortisol:

• Helps to slow the inflammatory response of the immune system
• Helps keep up cardiovascular function and blood pressure
• Helps balance the effects of insulin when breaking down sugar for energy
• Helps to regulate the metabolism of carbohydrates, proteins and fats

Since cortisol is so detrimental to health, the amount of cortisol made by the adrenal glands is balanced exactly. The failure to make the right levels of cortisol can happen for various reasons. The problem could be the result of a disorder of the adrenal glands, or primary adrenal insufficiency, or because of inadequate secretion of ACTH by the pituitary gland, or secondary adrenal insufficiency.

Symptoms of Addison’s Disease

Symptoms of Addison’s disease generally develop slowly, many times over several months and could include:

• Decreased appetite and weight loss
• Fatigue and muscle weakness
• Hyperpigmentation, or darkening of the skin
• Low blood pressure, and sometimes fainting
• Love blood sugar or hypoglycemia
• Craving for salt
• Irritability
• Joint or muscle pains
• Vomiting, nausea or diarrhea
• Depression
• Loss of body hair or sexual dysfunction for women

Acute Adrenal Failure or Addisonian Crisis

There are instances, however, where the symptoms and signs of the disease can appear quickly. In this situation, the symptoms sand signs can also include:

• Severe diarrhea and vomiting which leads to dehydration
• Pain in abdomen, legs or lower back
• Loss of consciousness
• High potassium or hyperkalemia

When to Visit Your Doctor

Visit your doctor if you have symptoms and signs which happen commonly in people with Addison’s disease like:

• Severe fatigue
• Hyperpigmentation
• Unintentional weight loss
• Fainting or dizziness
• Cravings for salt
• Gastrointestinal problems like vomiting, abdominal pain and nausea
• Joint or muscle pains

Your doctor will be able to figure out if you have Addison’s disease or another medical condition.

Causes & Risk Factors of Addison’s Disease

Small organs found near the top of each kidney that release hormones are known as the adrenal glands. They are comprised of an inner portion known as the medulla and the outer portion known as the cortex.

The cortex makes three types of hormones:

• Mineralocorticoid hormones like aldosterone, regulate potassium and sodium balance
• Glucocorticoid hormones like cortisol keep up sugar or glucose control and lower the immune response, plus help the body react to stress
• Sex hormones (androgens for males and estrogen for females) affect sex drive and sexual development

If the adrenal cortex is damaged, it can cause Addison’s disease. This damage makes the cortex produce hormone levels which are too low, causing low cortisol symptoms.

This type of damage can be caused by:

• Infections like tuberculosis, fungal infections or HIV
• The immune system will make the mistake of attacking the adrenal glands
• Tumors
• A hemorrhage inside the adrenal glands

Risk factors of other autoimmune diseases include:

• Dermatitis herpetiformis
• Chronic thyroiditis
• Hyperparathyroidism
• Grave’s disease
• Myasthenia gravis
• Hypopituitarism
• Testicular dysfunction
• Pernicious anemia
• Vitiligo
• Type 1 diabetes

Adrenal insufficiency could also be caused by certain rare genetic defects.

Diagnosis of Addison’s Disease

Your doctor will ask you about your health to diagnose Addison’s disease, such as if you’ve had cancer or if you have HIV or any history in your family of Addison’s disease. You will also go through a physical exam so your doctor can check for changes in the color of your skin, check blood pressure and look for dehydration signs.

Your doctor could also order tests:

• ACTH stimulation test – this checks how your hormone levels react to stress.
• Blood tests check for low sodium levels or high potassium. Your ACTH and cortisol levels might be checked as well.
• Imaging tests like an MRI, orCT scan to look for any damage to the adrenal glands.

Treatments for Addison’s Disease

All treatments will involve hormone replacement therapy so that the levels of steroid hormones that aren’t being produced can be fixed. Some options include:

• Corticosteroid injections. If you are ill and vomiting and you can’t keep oral injections down, this is a good alternative.
• Oral corticosteroids. You could be prescribed fludrocortisone to replace aldosteroine. Prednisone, hydrocortisone or cortisone acetate could be used to replace cortisol.
• Androgen replacement therapy. For androgen deficiency in women, you could be prescribed dehydroepiandrosterone. Studies have suggested that this could improve your overall sense of well-being, sexual satisfaction and libido.

A good amount of sodium is recommended, especially when the weather is hot, during a lot of exercise or if you have gastrointestinal problems. Your doctors could also temporarily increase you dosage if you are facing a stressful situation like an infection, operation or minor illness.

Surgery & Injections

People who have chronic adrenal insufficiency that need surgery with general anesthesia are treated with injections of saline and hydrocortisone. Injections start on the evening prior to surgery and keep going until the patient is completely awake and able to take oral medication. The dosage will be adjusted until the maintenance dosage given prior to surgery is achieved.

Pregnancy

Women who have primary adrenal insufficiency and get pregnant are treated with standard replacement therapy. If vomiting and nausea in the earlier stages of pregnancy interfere with oral medication, injections of the hormone could be needed. Treatment during delivery is similar to patients who need surgery. Following delivery, the dosage is slowly tapered.And the regular doses of fludrocortisone acetate and hydrocortisone orally are reached by around ten days after birth.