Addison’s disease, also known as primary adrenal insufficiency or hypocortisolism, is a rare condition in which the adrenal glands do not produce enough of certain essential hormones, particularly cortisol and often aldosterone. The adrenal glands are small glands located above the kidneys. Cortisol helps the body respond to physical and emotional stress, maintain blood pressure and blood glucose levels, and regulate metabolism and the immune system. Aldosterone helps maintain the body’s balance of sodium, potassium, fluids, and blood pressure. Addison’s disease usually develops gradually and may be difficult to recognize in its early stages because its symptoms can resemble those of other conditions. Without appropriate treatment, however, adrenal insufficiency can become life-threatening. A sudden and severe worsening of symptoms, known as an adrenal crisis, requires immediate medical attention.
An adrenal crisis is a medical emergency that can occur when cortisol levels become critically low. It may be triggered by severe infection, injury, surgery, significant physical stress, or suddenly stopping corticosteroid medication in people who depend on it. Warning signs can include:
Severe weakness
Severe abdominal, lower back, or leg pain
Persistent vomiting or diarrhea
Severe dehydration
Very low blood pressure
Confusion or loss of consciousness
Severe dizziness or fainting
Low blood sugar
Abnormal heart rhythm caused by electrolyte disturbances
An adrenal crisis can be life-threatening. Anyone with known or suspected adrenal insufficiency who develops severe symptoms should seek emergency medical care immediately.
Addison’s disease occurs when the outer part of the adrenal glands, called the adrenal cortex, is damaged and can no longer produce sufficient hormones. The most common cause in many countries is an autoimmune reaction, in which the body’s immune system mistakenly attacks the adrenal cortex. Other possible causes include:
Tuberculosis affecting the adrenal glands
Other infections that damage the adrenal glands
Adrenal gland cancer or metastatic cancer
Bleeding into the adrenal glands
Certain genetic or inherited disorders affecting adrenal function
Other conditions that damage or destroy the adrenal cortex
Addison’s disease is different from secondary adrenal insufficiency, in which inadequate production of adrenocorticotropic hormone (ACTH) by the pituitary gland leads to reduced cortisol production.
Addison’s disease is generally a lifelong condition, but appropriate hormone replacement therapy can control symptoms and help people lead active lives.
Treatment may include replacement of:
Cortisol, usually with hydrocortisone; prednisolone or other corticosteroids may be used in some circumstances.
Aldosterone, usually with fludrocortisone, when aldosterone deficiency is present.
The required medication dose may need to be temporarily increased during periods of physical stress, such as fever, significant illness, injury, or surgery. Patients should follow an individualized sick-day management plan provided by their healthcare professional. People with adrenal insufficiency are often advised to carry medical identification stating that they have adrenal insufficiency and may need emergency corticosteroid treatment.
People living with Addison’s disease should work closely with their healthcare provider and take their prescribed medicines consistently. Helpful measures may include:
Never stopping corticosteroid medication suddenly unless specifically instructed by a healthcare professional.
Following the prescribed sick-day or emergency steroid plan.
Keeping an adequate supply of medication available.
Carrying medical identification indicating adrenal insufficiency.
Discussing emergency injectable corticosteroid use with a healthcare professional when appropriate.
Maintaining adequate fluid intake, particularly during illness or in hot weather.
Following medical advice regarding salt intake and physical activity.
Consult a healthcare professional if you experience persistent unexplained fatigue, weight loss, loss of appetite, skin darkening, low blood pressure, salt cravings, or recurrent gastrointestinal symptoms. If you have known or suspected Addison’s disease and develop severe vomiting or diarrhea, extreme weakness, confusion, fainting, severe abdominal pain, or very low blood pressure, seek emergency medical care because these may be signs of an adrenal crisis.
Endocrinologist- An endocrinologist specializes in disorders involving hormones and glands, including adrenal insufficiency. Depending on the underlying cause and complications, other healthcare professionals may also be involved in treatment.
Addison’s disease usually cannot be cured when the adrenal glands have been permanently damaged. However, hormone replacement treatment can replace the hormones the body lacks and effectively control the condition.
Untreated Addison’s disease can become life-threatening. An adrenal crisis is a medical emergency that requires prompt treatment with corticosteroids and supportive care.
Skin darkening, or hyperpigmentation, can occur because the body produces increased amounts of ACTH when cortisol levels are low. ACTH is produced from a precursor molecule that also gives rise to melanocyte-stimulating activity, contributing to increased skin pigmentation.
With appropriate hormone replacement, regular medical follow-up, and proper management during illness or other physical stress, many people with Addison’s disease can lead active and fulfilling lives.
Illness can increase the body’s need for cortisol. Follow the sick-day or emergency medication instructions provided by your doctor. If you cannot keep oral medication down because of repeated vomiting, or if severe symptoms develop, seek urgent medical care.
Addison’s disease is a type of primary adrenal insufficiency, meaning the adrenal glands themselves are damaged and cannot produce sufficient hormones. Adrenal insufficiency can also result from problems involving the pituitary gland or from prolonged corticosteroid use.
Medical Disclaimer: This article is intended for general educational purposes and should not replace professional medical advice, diagnosis, or treatment. If you have symptoms suggestive of Addison’s disease or experience signs of an adrenal crisis, consult a qualified healthcare professional or seek emergency medical care.