Authors: Sidra Ali / Editor: Nikki Abela / Codes: EnC1, EnP1, SLO1 / Published: 26/07/2022

The case:

John is a 30-year-old male who has been wheeled into resus. He has been at home vomiting for two days and now has a reduced GCS. He is tachycardic and hypotensive and the sepsis team have started a screen on him. You run a blood gas which shows he has a blood glucose of 1.9 mmol/L, a sodium of 126 mmol/L and a potassium of 6 mmol/L. You start to suspect he may have an adrenal crisis, a.k.a Addisonian crisis.

Addison’s disease otherwise known as primary adrenal insufficiency or hypoadrenalism is a relatively rare disorder. Almost 9000 people in the UK have been diagnosed with this disorder and it can affect people of any age, although it mostly occurs in women and in those between the ages of 30-50.[1]

Although the 19th century physician Thomas Addison diagnosed the disease more than 150 years ago, the disease remains under diagnosed, leading to unnecessary morbidity and mortality. In fact, children with adrenal insufficiency have a higher risk of dying than those with other lifelong conditions.[2]

Addison’s disease is characterised by inadequate production of the steroid hormones cortisol and aldosterone by the adrenal cortex. People that suffer from this condition can develop an acute deterioration in the form of an ‘Addisonian Crisis’. In fact, at least one-fifth of patients with chronic adrenal insufficiency will have more than one adrenal crisis during their lifetime.[3] It is important to note that those with previously undiagnosed Addison’s disease may present to A&E in the form of an Addisonian crisis. There are a number of possible precipitating factors for this condition, including:

  • Infections 
  • Dehydration
  • Surgery
  • Trauma
  • Physiological stress/pain

Diagnosis

There is no fixed consensus on diagnostic criteria as most symptoms are non-specific:

  • hypotension
  • delirium
  • reduced consciousness
  • acute abdominal pain
  • nausea/vomiting 
  • headache 
  • fever 
  • muscle weakness
  • weight loss
  • loss of appetite 
  • muscle cramps 

Blood test results:

  • Hyponatraemia
  • Hyperkalaemia 
  • Hypoglycaemia                                                                                                         
  • Acute kidney injury

Investigations

  • FBC, U+Es & CRP
  • VBG
  • CXR, WTU & blood cultures (if sepsis suspected)

In undiagnosed adrenal insufficiency:

  • Paired sample of cortisol and ACTH before starting hydrocortisone (if this will not delay the hydrocortisone treatment

Treatment

It is vital that blood investigations are performed BEFORE commencing treatment. It is equally important to start treatment immediately if adrenal crisis is clinically suspected. 

Adults: Administer parenteral hydrocortisone 100mg stat (IM preferable) and repeat 6 hourly until the patient is haemodynamically stable and clinical improvement (alternative 200mg/24hrs by continuous IV infusion).  

Infants and children: Administer initial parenteral injection of 50mg hydrodortisone/m2 (usually 25mg in infants and 50mg in children) followed by 50mg/24h in infants and 100mg/24h in children.

Administer 1 litre IV 0.9% saline stat (adjust for infants and children), and continue saline resuscitation at an appropriate rate until haemodynamic stability and correction of any electrolyte disturbance and AKI. Some patients may require intensive care support to maintain their haemodynamic status without increasing their sodium too quickly – i.e. in some patients a bolus of saline and rapid fluid resuscitation may be detrimental and a more cautious approach may be needed.

Monitor U&E at least 12 hourly during initial resuscitation and continue regular monitoring until any hyponatraemia, hyperkalaemia or renal impairment are corrected

Identify and treat any precipitating cause for Addisonian crisis.

References

  1. Addison’s disease NHS uk.
  2. Action needed on high mortality in children with adrenal insufficiency. The University of Manchester. 2021.
  3. Allolio B. Extensive expertise in endocrinology. Adrenal crisis. Eur J Endocrinol. 2015 Mar;172(3):R115-24.