Adrenal Disorders

What the adrenal glands do

Each adrenal gland has two parts:

Cortisol production is controlled by ACTH from the pituitary gland, so pituitary problems also affect the adrenals.

Types of adrenal disorders

Adrenal insufficiency and Addison's disease

When the adrenals make too little cortisol, and often too little aldosterone, symptoms develop gradually:

• fatigue, weakness and weight loss
• dizziness on standing and low blood pressure
• salt craving
• nausea, vomiting and abdominal pain
• darkening of the skin, especially in creases, scars and the gums (in primary disease)

Addison's disease is primary adrenal insufficiency, most often autoimmune. Secondary adrenal insufficiency follows pituitary disease or, most commonly, stopping long-term steroid medicines (such as prednisone) too quickly, because the adrenals have been suppressed.

An adrenal crisis is a life-threatening drop in cortisol, often triggered by infection, vomiting, injury or surgery. It causes severe weakness, vomiting, low blood pressure and confusion, and needs immediate hydrocortisone injection.

Cushing's syndrome

Cushing's syndrome results from too much cortisol. The commonest cause is long-term steroid medicine. Other causes are a pituitary tumor making ACTH (Cushing's disease), an adrenal tumor, or rarely ACTH from a tumor elsewhere. Features include:

• weight gain in the face (round face), upper back and abdomen, with thin arms and legs
• wide purple stretch marks, thin skin and easy bruising
• muscle weakness, especially getting up from a chair
• high blood pressure, diabetes, osteoporosis
• mood changes, irregular periods, excess hair

Primary aldosteronism (Conn's syndrome)

In hyperaldosteronism, the adrenals make too much aldosterone, causing the body to retain salt and lose potassium. It is a common cause of high blood pressure that is resistant to treatment. Low potassium can cause weakness, cramps and frequent urination, but many people have normal potassium. Causes are a single adrenal adenoma (curable with surgery) or overgrowth of both glands (treated with medicines).

Pheochromocytoma

A pheochromocytoma is a usually benign tumor of the adrenal medulla that releases bursts of adrenaline. Typical attacks combine headache, sweating and a pounding heart, with very high or fluctuating blood pressure, anxiety, tremor and pallor. Some are linked to inherited syndromes, so genetic testing is recommended.

Congenital adrenal hyperplasia

Congenital adrenal hyperplasia (CAH) is an inherited enzyme deficiency, most often 21-hydroxylase, in which the adrenals cannot make enough cortisol and aldosterone and make too many androgens. Severe forms are detected by newborn screening in the US and can cause salt-wasting crises in the first weeks of life. Milder forms appear later with early puberty, excess hair, acne or irregular periods.

Adrenal tumors and incidentalomas

Adrenal lumps are often found by chance on CT scans done for other reasons. Most are non-functioning adrenal adenomas. All should be tested for hormone overproduction, and larger or irregular lumps assessed for adrenal cancer, which is rare.

Diagnosis

Testing depends on the suspected disorder:

• Adrenal insufficiency: early morning cortisol, ACTH level and an ACTH (cosyntropin) stimulation test.
• Cushing's syndrome: late-night salivary cortisol, 24-hour urine cortisol, or an overnight dexamethasone suppression test.
• Primary aldosteronism: aldosterone-to-renin ratio, followed by confirmatory tests and sometimes adrenal vein sampling.
• Pheochromocytoma: plasma or urine metanephrines.
• CAH: 17-hydroxyprogesterone.
• Imaging: CT or MRI of the adrenals or pituitary after hormone tests point to a cause.

Treatment

• Adrenal insufficiency: daily hydrocortisone (and fludrocortisone in Addison's disease), with extra doses during illness ("sick day rules"), an emergency injection kit and medical alert ID.
• Cushing's syndrome: reducing steroid medicines gradually, surgery to remove the pituitary or adrenal tumor, or medicines that block cortisol production.
• Primary aldosteronism: removing a single adenoma by keyhole surgery, or mineralocorticoid receptor blockers such as spironolactone.
• Pheochromocytoma: alpha-blocker medicine for 1 to 2 weeks, then surgical removal.
• CAH: glucocorticoid and mineralocorticoid replacement.

Never stop long-term steroid medicine suddenly. Doses must be reduced gradually under medical guidance.

Frequently asked questions

What are the symptoms of adrenal gland problems?

It depends on the hormone involved. Too little cortisol causes fatigue, weight loss, dizziness and salt craving. Too much cortisol causes weight gain, stretch marks and high blood pressure. Too much aldosterone causes resistant high blood pressure. Too much adrenaline causes attacks of headaches, sweating and palpitations.

What is the most common adrenal disorder?

Primary aldosteronism is one of the most common, found in a significant share of people with hard-to-treat high blood pressure. Adrenal insufficiency after stopping steroid medicines is also common. Harmless adrenal adenomas found on scans are very common.

Is adrenal fatigue a real condition?

"Adrenal fatigue" is not a recognized medical diagnosis, and there is no evidence that stress wears out the adrenal glands. True adrenal insufficiency is a real, testable condition. Taking adrenal supplements without a diagnosis can be harmful.

Can adrenal disorders be cured?

Many can. Removing a pheochromocytoma, an aldosterone-producing adenoma or a cortisol-producing tumor can cure the condition. Addison's disease and CAH need lifelong hormone replacement but are very manageable.