Addison's disease Flashcards

1
Q

What is Addison’s disease?

A
  • 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)
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2
Q

Describe the pathway involved in adrenal function

A
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3
Q

What are the causes of primary adrenocortical insufficiency?

A
  • 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)
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4
Q

What is the commonest cause of secondary adrenocortical insufficiency?

A
  • Chronic steroid use
  • It suppresses the pituitary-adrenal axis
  • This only becomes apparent when steroids are withdrawn
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5
Q

What are the other rarer causes of secondary adrenocortical insufficiency?

A
  • Hypothalamic-pituitary disease leading to decreased ACTH production
    • Mineralocortioid production remains intact
    • And there is no hyperpigmentation as ACTH production is decreased
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6
Q

What are the symptoms od Addison’s disease?

A
  • 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
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7
Q

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

A
  • Shock
    • Low BP
    • Tachycardia
  • High temperature
  • Coma
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8
Q

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

A
  • Sodium and potassium (low and high respectively)
  • Low glucose
  • Ureamia
  • Hypercalcaemia
  • Eosinophilia
  • Anaemia
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9
Q

What is the diagnostic test for Addison’s disease?

A
  • 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
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10
Q

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

A
  • AXR/CXR
    • For signs of TB or past TB
  • Adrenal CT
    • For TB
    • Histoplasma
    • Metastatic disease
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11
Q

What is the treatment for Addison’s disease?

A
  • 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)
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12
Q

What advice should be given for steroid use?

A
  • 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
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13
Q

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

A
  • 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
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14
Q

What is Addisonian crisis?

A
  • 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.
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15
Q

What are the signs and symptoms of Addisonian crisis?

A
  • Shock
    • Raised HR
    • Vasoconstriction
    • Postural hypotension
    • Oligouria
    • Weakness
    • Confused
    • Comatose
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16
Q

What are the precipitating factors of Addisonian crisis?

A
  • 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
17
Q

How is an Addisonian crisis managed?

A
  • 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
18
Q

What is the continuing treatment for Addisonian crisis?

A
  • 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