Overview
An adrenal crisis is a life-threatening condition associated with an acute deficiency in steroid hormones, namely the glucocorticoid cortisol and, to a lesser extent, the mineralocorticoid aldosterone. It requires immediate emergency treatment.
To help make sense of this chapter, it may be helpful to refer to Endocrine and Metabolic Physiology.
Causes
- Infections (e.g. pneumonia, gastrointestinal illness etc.)
- Abrupt steroid withdrawal:
- This can lead to an adrenal crisis in a patient without Addison’s disease. Exogenous corticosteroid use can lead to secondary adrenal insufficiency. This is why corticosteroids are tapered off. In simple terms, it allows the adrenal glands to ‘wake back up’ and start producing cortisol again.
- Peri-operatively
- Physiological stress/pain
- Dehydration
- Emotional distress
- Hypoglycaemia in patients with diabetes
- Haemorrhage – Waterhouse-Friderichsen syndrome:
- This is due to disseminated intravascular coagulopathy leading to haemorrhage into the adrenal glands, often as a result of infection with Neisseria meningitidis.
Presentation
It is important to remember that adrenal crises can happen in people without Addison’s disease (e.g. those who suddenly stop taking long-term corticosteroids). Features may be:
- Haemodynamic instability – hypotension and tachycardia
- Malaise
- Fatigue
- Vague abdominal pain – acutely worsening, this suggests an adrenal crisis
- Low-grade fever
- Muscle pains and cramps
- Confusion
- Dehydration
- Loss of consciousness
Differential Diagnoses
Diabetic ketoacidosis (DKA)
- Blood sugars and ketones are raised in DKA, whereas in primary adrenal insufficiency, blood sugars may be low
Investigations
All patients
Treatment should be initiated immediately based on physical features. Do not delay treatment while waiting for test results.
Other investigations
- FBC:
- White cells may be elevated if there is an underlying infection
- TFTs:
- Performed as a baseline
- U&Es, ABG/VBG, and glucose:
- May show hyponatraemia
- May show hyperkalaemia
- May show hypoglycaemia – characteristic in children
- May show slight hypercalcaemia
- May show normal ion gap metabolic acidosis
Diagnosis
Diagnosis is clinical. Investigations are used to confirm the diagnosis once treatment has been initiated.
Management
All patients
- 1st-line: IM or IV hydrocortisone + IV rehydration with normal saline + treat underlying cause
- The doses of hydrocortisone used in an adrenal crisis are enough to exert mineralocorticoid effects, so fludrocortisone is not necessary.
Prevention
- Steroids must not be stopped suddenly if they have been used for >2 weeks
- Perioperative ‘stress doses’ of steroids may be given to mitigate the risk of adrenal crises arising
Complications and Prognosis
Complications
- Circulatory collapse
- Death
Prognosis
- The prognosis is generally better in those who have suffered an adrenal crisis secondary to exogenous glucocorticoid use compared to those with adrenal insufficiency.
