Adrenal Insufficiency: A Patient’s Guide to Symptoms, Causes and Management

Overview

Adrenal insufficiency is a rare endocrine condition that occurs when your adrenal glands — the small, triangle-shaped glands sitting on top of each kidney — do not produce enough of two critical hormones: cortisol and aldosterone. Cortisol is your body’s primary stress hormone, which helps regulate metabolism, immune function, blood pressure, and your response to illness or injury. Aldosterone controls your body’s balance of sodium and potassium, which in turn regulates blood pressure and fluid balance. There are two main types of adrenal insufficiency: primary adrenal insufficiency (also called Addison’s disease), which happens when the adrenal glands themselves are damaged, and secondary adrenal insufficiency, which occurs when the pituitary gland in your brain fails to send the signal (adrenocorticotropic hormone, or ACTH) that tells your adrenal glands to make cortisol. If left untreated, adrenal insufficiency can lead to a life-threatening complication called adrenal crisis.

Signs & Symptoms

Symptoms of adrenal insufficiency often develop slowly over months or years, making them easy to mistake for other common conditions like depression or chronic fatigue syndrome. Common signs include persistent fatigue and muscle weakness, unintentional weight loss, low blood pressure (especially when standing up, called orthostatic hypotension), and darkening of the skin (hyperpigmentation) — most noticeable on the elbows, knees, knuckles, and inside of the cheeks, which is unique to primary adrenal insufficiency. Many people also experience salt cravings, nausea, vomiting, abdominal pain, joint or muscle pain, and irritability or depression. Symptoms of an adrenal crisis, a medical emergency, include sudden severe weakness, confusion, severe abdominal pain, low blood pressure, fever, and loss of consciousness.

Causes & Risk Factors

Primary adrenal insufficiency (Addison’s disease) is most often caused by autoimmune disease, where your body’s immune system mistakenly attacks the adrenal glands. Other causes include infections (such as tuberculosis or fungal infections that spread to the adrenal glands), bleeding into the adrenal glands, cancer that spreads to the adrenal glands, or genetic disorders that affect adrenal function. Secondary adrenal insufficiency is usually caused by problems with the pituitary gland, such as pituitary tumors, surgery or radiation to the pituitary, or sudden stopping of corticosteroid medications (like prednisone) that you have taken for a long time, which suppresses your body’s natural cortisol production. Risk factors for adrenal insufficiency include having another autoimmune condition (like type 1 diabetes or autoimmune thyroid disease), a history of pituitary surgery or radiation, or long-term use of high-dose steroid medications.

Diagnosis

Diagnosing adrenal insufficiency starts with a review of your symptoms, medical history, and a physical exam. Your provider will likely order blood tests to measure your cortisol and ACTH levels, often first thing in the morning when cortisol is highest. If initial tests suggest adrenal insufficiency, you may need an ACTH stimulation test, where you are given a synthetic form of ACTH and your cortisol levels are measured 30 and 60 minutes later to see how well your adrenal glands respond. Additional tests may include imaging of your adrenal glands or pituitary gland to look for damage or tumors, and tests to check for underlying causes like autoimmune disease or infections.

Treatment & Management

There is no cure for adrenal insufficiency, but it is very manageable with lifelong hormone replacement therapy. For primary adrenal insufficiency, you will take two types of medications: hydrocortisone or prednisone to replace cortisol, and fludrocortisone to replace aldosterone. For secondary adrenal insufficiency, you will only need cortisol replacement, since aldosterone production is usually not affected. Your provider will work with you to find the right dose of medication, which may need to be adjusted during times of illness, stress, or surgery, when your body needs more cortisol. It is critical to never skip your medication, as this can trigger adrenal crisis.

Living With It

With proper treatment and monitoring, most people with adrenal insufficiency can live full, active lives. Regular follow-up appointments with your endocrinologist every 6 to 12 months are important to check your hormone levels, adjust your medication dose, and screen for complications like osteoporosis or high blood pressure from over-replacement of steroids. You will need to carry an emergency injection of hydrocortisone with you at all times, in case of adrenal crisis, and make sure you and your loved ones know how to use it correctly. It is also helpful to keep extra medication on hand, eat a balanced diet with adequate salt (if recommended by your provider), and avoid extreme physical or emotional stress when possible.