An Addisonian crisis, also known as an adrenal crisis, is a severe and potentially life-threatening condition that arises due to acute adrenal insufficiency. It is a rare condition with an estimated prevalence of around 600 per million, but it does present to the Emergency Department, and the consequences can be catastrophic if the diagnosis is missed. It is more common in women than men and most commonly occurs between the ages of 30 and 50. Understanding the pathophysiology, clinical presentation, and management strategies is crucial for medical professionals to address this emergency effectively.
Pathophysiology
Adrenal insufficiency occurs when the adrenal glands fail to produce adequate amounts of cortisol and aldosterone. Cortisol, a glucocorticoid, is essential for stress response, metabolism, and immune function, while aldosterone, a mineralocorticoid, regulates sodium and potassium balance.
Primary adrenal insufficiency (Addison’s disease) results from direct damage to the adrenal cortex. The underlying causes of Addison’s disease include autoimmune adrenalitis (80% of cases), bilateral adrenalectomy, Waterhouse-Friderichson syndrome (haemorrhage into adrenal glands) and tuberculosis.
Secondary adrenal insufficiency stems from insufficient adrenocorticotropic hormone (ACTH) production by the pituitary gland, which may be due to pituitary tumours, surgery, or irradiation
In an Addisonian crisis, the abrupt deficiency of these hormones leads to severe electrolyte imbalances and cardiovascular collapse. The commonest cause of an Addisonian crisis in patients with Addison’s disease is the deliberate or accidental withdrawal of steroid therapy. Other precipitating factors include:
- Infection
- Trauma
- Myocardial infarction
- Cerebral infarction
- Asthma
- Hypothermia
- Alcohol abuse
Clinical features
The clinical features of primary adrenal insufficiency include:
- Weakness and lethargy
- Hypotension (notably orthostatic hypotension)
- Nausea and vomiting
- Weight loss
- Reduced axillary and pubic hair
- Depression
- Hyperpigmentation (palmar creases, buccal mucosa and exposed areas more commonly affected)
The onset of an Addisonian crisis is often sudden and can be dramatic. The main features are usually hypoglycaemia and shock (tachycardia, peripheral vasoconstriction, hypotension, altered conscious level, and coma). The shock is often unresponsive to fluid resuscitation alone. Other features of an Addisonian crisis include:
- Electrolyte imbalance: hyponatraemia, hyperkalaemia, and hypoglycaemia are common due to aldosterone deficiency and impaired gluconeogenesis.
- Gastrointestinal symptoms: nausea, vomiting, abdominal pain, and diarrhoea can occur, sometimes mimicking acute abdomen.
- Neurological manifestations: Confusion, lethargy, and even coma may develop due to hypoglycaemia and electrolyte disturbances.
- General symptoms: severe weakness, fatigue, and dehydration are typically present.
Diagnosis and investigations
Timely diagnosis of an Addisonian crisis involves a combination of clinical suspicion and laboratory testing. Bloods should be taken before commencing treatment.
The classical biochemical features of Addison’s disease are as follows:
- Increased ACTH levels (rise in an attempt to stimulate the adrenal glands)
- Elevated serum renin level
- Hyponatraemia
- Hyperkalaemia
- Hypercalcaemia
- Hypoglycaemia
- Metabolic acidosis
Investigations that can confirm the diagnosis include:
- Synacthen test
- Plasma ACTH level
- Plasma renin level
- Adreno-cortical antibodies
Management of an Addisonian crisis
The immediate management of an Addisonian crisis focuses on reversing hypotension, correcting electrolyte imbalances, and replacing deficient hormones. Key interventions include:
Corticosteroid replacement:
- Administer a stat dose of parenteral hydrocortisone, e.g. 100 mg intramuscular bolus
- This should be followed by 100 mg every 6-8 hours until the patient is stable and clinical improvement is evident
- Dexamethasone can be used if hydrocortisone is unavailable.
Fluid resuscitation:
- Intravenous fluids should be commenced immediately
- Administer 1 litre of 0.9% saline stat (adjust for infants and children)
- Continue fluid resuscitation at an appropriate rate until the patient is haemodynamically stable
Electrolyte management:
- Sodium and potassium levels should be carefully monitored and corrected
- Treat hypoglycaemia with intravenous dextrose
- An insulin and dextrose infusion may be necessary in cases of severe hyperkalaemia
- Intensive care support may be required to maintain the patient’s haemodynamic status without increasing their sodium too quickly
- U&Es should be measured at least 12 hourly during initial resuscitation, and regular monitoring will be necessary until the patient is stable
Address underlying causes:
- Any precipitating causes or factors, such as infection, should be identified and treated
- Take blood cultures, urine cultures and sputum for MC&S (preferably before the administration of IV antibiotics)
Long-term management
An endocrinologist should manage Addison’s disease. Typically, patients require hydrocortisone, fludrocortisone, and dehydroepiandrosterone. Some patients also require thyroxine if hypothalamic-pituitary disease is present.
Treatment is lifelong, and patients should carry a steroid card and a MedicAlert bracelet and be aware of the possibility of an Addisonian crisis. It is of the utmost importance to educate patients about the importance of adherence to corticosteroid therapy, recognising early signs of adrenal insufficiency, and adjusting doses during illness or stress.
Header image used on licence from Shutterstock
Thank you to the joint editorial team of www.mrcemexamprep.net for this article.
