Adrenal insufficiency

Adrenal insufficiency, also sometimes called adrenal dysfunction or Addison’s disease, is the name given to the underproduction of steroid hormones by the adrenal cortex of the adrenal glands (Nematollahi and Arafah, 2023). As well as lesser hormones such as dehydroepiandrosterone, the adrenal cortex produces two main classes of steroid hormones:

• glucocorticoids (eg cortisol)
• mineralocorticoids (eg aldosterone) (Nematollahi and Arafah, 2023)

The production of steroid hormones is primarily regulated by the hypothalamic–pituitary–adrenal (HPA) axis (glucocorticoids) and renin–angiotensin system (mineralocorticoids). Since these hormones play a role in the management of metabolic activity, as well as water and electrolyte balance in the body, the signs and symptoms of adrenal insufficiency are broadly related to these functions.

Cortisol enables the body to respond to stress and plays a role in the management of:

• blood pressure
• blood glucose
• inflammation
• metabolism

Aldosterone, the other main adrenal hormone, plays a role in the management of sodium and

The signs and symptoms of adrenal insufficiency occur in response to the body’s reduced ability to manage metabolic activity. The signs and symptoms of adrenal insufficiency vary in severity and include:

• fatigue
• gastrointestinal issues
• weight loss
• craving for salt
• hypotension (often postural)
• dizziness and/or syncope
• arthralgia and myalgia
• loss of pubic and axillary hair in women (Nematollahi and Arafah, 2023)

The most serious complication of adrenal insufficiency is adrenal crisis, symptoms of which can include:

• sudden, severe lower back, abdomen or leg pain
• vomiting and diarrhoea
• weakness
• confusion
• loss of consciousness
• fatigue
• severe dehydration (NHS, 2021)

The causes of adrenal insufficiency are divided into primary, secondary and tertiary causes. The most common causes of primary adrenal insufficiency include:

• Addison's disease (an autoimmune disease)
• congenital adrenal hyperplasia
• bilateral adrenalectomy (usually for malignancy)
• adrenal haemorrhage (traumatic and non-traumatic, eg from anticoagulant use, sepsis, pregnancy)

Secondary adrenal insufficiency occurs as a result of conditions affecting the production of hormones, primarily adrenocorticotropin, which acts on the adrenal glands, causing them to make cortisol. Examples include pituitary diseases or tumours and their treatment (ie surgery and radiotherapy).

Tertiary adrenal insufficiency starts in the hypothalamus, which produces corticotropin-releasing hormone, a hormone that prompts the pituitary gland to make adrenocorticotropin. Tertiary adrenal insufficiency may result from suppression of the hypothalamic–pituitary–adrenal axis, for example from steroids and rarely treatment of primary brain or nasopharyngeal tumours with radiotherapy (Hahner et al, 2021; National Institute for Health and Care Excellence (NICE), 2023)

Blood tests for diagnosis of adrenal insufficiency include:

• morning serum cortisol levels
• adrenocorticotropin
• urea and electrolytes (low sodium and high potassium)
• blood glucose levels (borderline or low) (NICE, 2020a; Nematollahi and Arafah, 2023)

In the absence of a definitive result, other tests might include:

• adrenocorticotropin stimulation testing
• plasma renin activity (Nematollahi and Arafah, 2023)

Once a person has been diagnosed and is taking steroids, patients are advised to carry an emergency card (NHS England, 2020; Society for Endocrinology, 2023) or MedicAlert bracelet (Nematollahi and Arafah, 2023), which alerts healthcare professionals to their condition, as early identification in an emergency can be lifesaving.

The aim of treatment for adrenal insufficiency includes improving the quality of life of those affected by reducing complications and symptoms. This involves replacing the usual physiological levels of glucocorticoids for the individual with:

• hydrocortisone
• prednisolone
• dexamethasone (rarely) (NICE, 2023)

The hormone replacement regimen should be tailored to closely mimic the diurnal cortisol secretion pattern and to the needs of the individual (Hahner et al, 2021). This may involve dividing daily doses to provide even levels of glucocorticoid throughout the day (NICE, 2020b).

Mineralocorticoid replacement may include the use of fludrocortisone as a replacement for aldosterone, while dehydroepiandrosterone may rarely be prescribed by specialist endocrinologists for androgen replacement therapy.

Nurses and other healthcare professionals have a role in educating affected individuals on the life-long nature of the treatment, and that they must follow the treatment regime closely in order to avoid a crisis (NICE, 2020b).