Addison's disease

Question 1

What is Addison’s disease?

Answer 1

• Addison’s disease, also known as primary adrenal insufficiency and hypocortisolism, is a long-term endocrine disorder in which the adrenal glands do not produce enough steroid hormones
• Destruction of the adrenal cortex leasd to glucocorticoid (cortisol) and mineralocorticoid (aldosterone) insufficiency
• Signs are volatile and inconsistent
• It maye be diagnosed as anorexia nervosa or viral infection in error (but K is decreased in the former and raised in Addison’s)

Question 2

Describe the pathway involved in adrenal function

Answer 2

Question 3

What are the causes of primary adrenocortical insufficiency?

Answer 3

• Autoimmunity (80% of the cause in UK)
• TB (commonest cause worldwide)
• Adrenal metastases
• Lymphoma
• Opportunistic infections in HIV
• Adrenal haemorrhage
• Waterhouse - Friderichsen syndrome
• Antiphospholipid syndrome
• Congenital (late onset congenital adrenal hyperplasia)

Question 4

What is the commonest cause of secondary adrenocortical insufficiency?

Answer 4

• Chronic steroid use
• It suppresses the pituitary-adrenal axis
• This only becomes apparent when steroids are withdrawn

Question 5

What are the other rarer causes of secondary adrenocortical insufficiency?

Answer 5

• Hypothalamic-pituitary disease leading to decreased ACTH production
  
  • Mineralocortioid production remains intact
  • And there is no hyperpigmentation as ACTH production is decreased

Question 6

What are the symptoms od Addison’s disease?

Answer 6

• Tanned
  
  • pigmented palmar creases &buccal mucosa
• Lean
• Tired
• Tearful
• Weak
• Anorexic
• Dizzy
• Faint
• Flu like myalgias and arthralgias
• Changes in mood
  
  • depression
  • psychosis
• GI
  
  • nausea/vomiting
  • unexplained abdominal pain
  • diarrhoea/constipation
• Postural hypotension
• Vitiligo

Question 7

What are the signs of critical deterioration in Addison’s disease?

Answer 7

• Shock
  
  • Low BP
  • Tachycardia
• High temperature
• Coma

Question 8

What tests should be done to diagnose Addison’s disease?

Answer 8

• Sodium and potassium (low and high respectively)
• Low glucose
• Ureamia
• Hypercalcaemia
• Eosinophilia
• Anaemia

Question 9

What is the diagnostic test for Addison’s disease?

Answer 9

• SynACTHen test - Short ACTH stimulation test
  
  • Check plasma cortisol before and half an hour after giving the patient synacthen IM
  • In healthy individuals, the cortisol level should increase
  • In Addison’s disease, both the cortisol and aldosterone levels are low, and the cortisol will not rise during the stimulation test
• ACTH test
  
  • In Addisons ACTH is abnormally high at 9am and low in secondary causes of Addisons
• 21 Hydroxylase adrenal autoantibodies (+ve in autoimmune diseases in 80%)
• Plasma renin and aldosterone to assess mineralocorticoid status

Question 10

What imaging tests should you carry out for Addison’s disease?

Answer 10

• AXR/CXR
  
  • For signs of TB or past TB
• Adrenal CT
  
  • For TB
  • Histoplasma
  • Metastatic disease

Question 11

What is the treatment for Addison’s disease?

Answer 11

• Replace steroids
  
  • HYDROCORTISONE DAILY
• Give mineralocorticoids to correct postural hypotension
  
  • FLUDROCORTISONE DAILY
• If there is a poor response then consider an associated autoimmune disease (check thyroid or do coeliac serology)

Question 12

What advice should be given for steroid use?

Answer 12

• Avoid giving steroids late as it may cause insomnia
• Avoid stopping steroids abrubtly
• Wear steroid bracelet declaring steroid use
• Add 5-10mg hydrocortisone to daily intake before strenuous activity or exercise
• Follow up yearly (BP and U&Es)
• Watch for autoimmune diseases (pernicious anaemia)
• Give out syringes and show the patient how to inject IM if they are vomiting and cannot take oral steroids

Question 13

What advise should be given to patients for exogenous steroid use?

Answer 13

• Replacement steroids are vital in those taing long term steroids when acutely unwell
• Adrenal insufficiency will develop with deadly hypovolaemic shock if additional steroids are not given
• Warn against abruptly stopping steriods
• Emphasise that prescribing doctors must know about steroid use –> give them a steroid card

Question 14

What is Addisonian crisis?

Answer 14

• It is a medical emergency and potentially life-threatening situation requiring immediate emergency treatment.
• It is a constellation of symptoms that indicate severe adrenal insufficiency caused by insufficient levels of the hormone cortisol.

Question 15

What are the signs and symptoms of Addisonian crisis?

Answer 15

• Shock
  
  • Raised HR
  • Vasoconstriction
  • Postural hypotension
  • Oligouria
  • Weakness
  • Confused
  • Comatose

Question 16

What are the precipitating factors of Addisonian crisis?

Answer 16

• Infection
  
  • When a patient with known Addison’s does not have replacement or an increased dose of steroids to cover stress such as infection
• Missed medication
  
  • Someone on long term steroids who has forgotten their tablets
• Bilateral adrenal haemorrhage
• Trauma
• Surgery

Question 17

How is an Addisonian crisis managed?

Answer 17

• Bloods
  
  • Cortisol
  • ACTH
  • U&Es
    
    • Potassium may be raised therefore check ECG and give calcium gluconate if needed)
  • Decreased sodium
    
    • Salt depletion should resolve with rehydration and steroids
• Hydrocortisone IV 100mg stat
• IV fluid bolus - 500ml 0.9 saline in 15 mins
  
  • repeat as necessary
• Monitor blood glucose
  
  • Hypoglycaemia is the danger
• Blood, urine and sputum culture
• Antibiotics if concerned about infection

Question 18

What is the continuing treatment for Addisonian crisis?

Answer 18

• Glucose IV may be needed if hypoglycaemic
• IV fluids to correct U&Es and clinical state
• Continue hydrocortisone 100mg/8hr IV or IM
  
  • Change to oral steroids after 72hrs if patients condition improves
• Flurocortisone
  
  • Used if the cause of crisis is adrenal disease but ask an expert
• Search for and treat the underlying cause ASAP
• Seek help from endocrinologist