Addison's disease Flashcards

1
Q

What is Addison’s disease?

A

Adrenal insufficiency. Reduced amounts of cortisol and aldosterone being produced.

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2
Q

What is the pathophysiology of Addison’s Disease?

A

Decreased production of adrenocortical hormones due to either destruction of the adrenal cortex or disruption of hormone synthesis. Infiltrating diseases (e.g. TB or metastasis) can also cause destruction of the adrenal medulla.

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3
Q

What are the 3 layers of the adrenal cortex?

A

Zona Glomerulosa

Zona Fasciculata

Zona Reticularis

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4
Q

What are the 3 adrenocortical hormones?

A
  • Aldosterone
  • Cortisol
  • Dehydroepiandrosterone
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5
Q

What is primary adrenal insufficiency?

A

Reduced secretion of adrenal hormones due to destruction or dysfunction of the adrenal gland

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6
Q

What is secondary adrenal insufficiency?

A

Inadequate pituitary adrenocorticotrophic hormone (ACTH) release and subsequent cortisol production.

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7
Q

What is the most common cause of primary hypoadrenalism in the UK?

A

Autoimmune destruction of the adrenal glands (80% of cases)

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8
Q

What are other primary causes of Addison’s disease?

A

Primary causes

  • TB (most prevalent cause in endemic countries)
  • Metastases (e.g. bronchial carcinoma)
  • Meningococcal septicaemia (Waterhouse-Friderichsen syndrome)
  • HIV
  • Antiphospholipid syndrome
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9
Q

What are secondary causes of Addison’s disease?

A

Secondary causes

  • Pituitary disorders (e.g. tumours, irradiation, infiltration)
  • Exogenous glucocorticoid therapy
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10
Q

What are symptoms of Addison’s disease?

A
  • Lethargy / Weakness
  • Anorexia / weight loss
  • Dizziness (less common)
  • Arthralgia / Myalgia (less common)
  • GI symptoms
    • Nausea & vomiting
    • Abdo pain
    • Constipation
  • Salt-craving (Hyponatraemia / Hyperkalaemia)

History should be directed at establishing the underlying cause, such as a known history of autoimmune disease, HIV infection, possible tuberculosis infection, and current medication use.

Sudden alterations in glycaemic control and recurrent hypoglycaemia in patients with type 1 diabetes mellitus may suggest autoimmune polyglandular syndrome type 2 with co-existing diabetes and Addison’s disease

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11
Q

What are the signs of Addison’s disease found on examination?

A
  • Mucosal & cutaneous hyperpigmentation
    • Of palmar creases, knuckles, scars & buccal mucosa
  • Hypotension (Postural)
  • Signs of autoimmunity
    • Vitiligo
    • Hashimoto’s thyroiditis
    • Pernicious anaemia
  • Loss of axillary and pubic hair
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12
Q

How can you distinguish primary adrenal insuffiency from secondary adrenal insufficiency from the presentation alone?

A

Primary adrenal insufficiency is associated with hyperpigmentation whereas secondary adrenal insufficiency is not.

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13
Q

What investigations are used to confirm a diagnosis of Addison’s disease?

A

1. Routine bloods (FBC, U&Es)

  • Don’t provide diagnosis but can be useful to confirm presence of anaema, hyperkalaemia, hyponatraemia, etc.

2. 9am serum cortisol

+ve result = LOW

  • Used in primary care or when Synacthen test unavailable

3. ACTH stimulation (Synacthen) test = Definitive investigation

4. Additional tests

  • ACTH test
  • Autoantibody test
  • Insulin hypoglycaemia test
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14
Q

How would you interpret the results of a 9am Serum Cortisol Test?

A
  • > 500 nmol/l = Normal - Addison’s very unlikely
  • 100-500 nmol/l = ACTH stimulation test should be performed
  • < 100 nmol/l = Abnormal
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15
Q

You perform U&E’s and VBG on a patient with Addison’s disease. What abnormalities may you find?

A
  • Hyperkalaemia
  • Hyponatraemia
  • Metabolic acidosis
  • Hyperglycaemia
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16
Q

How is Addison’s disease managed?

A
  1. Combination of glucocorticoid therapy and mineralocorticoid therapy:
  • Hydrocortisone: 20-30 mg per day, divided into 2 or 3 doses.
  • Fludrocortisone
  1. Androgen replacement (Dehydroepiandrosterone)
    * For women with reduced libido
17
Q

What patient advice is required when treating patients with steroids for Addison’s disease?

A
  • Advice that treatment is lifelong
  • Emphasise the importance of not missing glucocorticoid doses
  • Educate of the signs of Adrenal crisis & how to give IM adrenaline
  • Consider MedicAlert bracelets and steroid cards
  • Discuss sick day rules
18
Q

During significant stress such as infection, trauma, and surgery, what changes should be made to the medication of patients with adrenal insufficiency?

A

Double the dose of glucocorticoids (e.g. hydrocortisone)

Keep the dose of mineralocorticoids (e.g. fludrocortisone) the same

19
Q

What are 3 signs & 3 symptoms of Addisonian crisis?

A

Signs

  • Collapse
  • Shock
  • Pyrexia

Symptoms

  • Abdominal pain
  • Confusion
  • Nausea & vomiting
20
Q

What is MedicAlert ID?

A

A bracelet / necklace allowing healthcare professionals to access a 24-hour helpline for more information on a person’s condition in an emergency if they are not able to give information themselves.

21
Q

How is adrenal crisis managed?

A
  1. Hydrocortisone 100 mg IM / IV
  • Continued 6 hourly until the patient is stable
  • Oral replacement may begin after 24 hours and be reduced to maintenance over 3-4 days
  1. Normal saline (1L over 30-60 mins)

+/- dextrose (if hypoglycaemic)

  1. Treat any underlying causes
22
Q

What is a steroid treatment card?

A

A card that can fit into a wallet to alert healthcare professionals that the person is on steroid treatment which should not be stopped suddenly.

23
Q

What is an emergency crisis letter?

A

A letter for healthcare professionals that people with Addison’s disease can carry with them in case of medical emergency. Available in a variety of languages and found on the Addison’s Disease Self Help Group website.

24
Q

What are 3 causes of adrenal crisis?

A
  • Stress (e.g. sepsis / surgery) causing an acute exacerbation of chronic insufficiency (Addison’s, Hypopituitarism)
  • Adrenal haemorrhage (e.g. Waterhouse-Friderichsen syndrome)
  • Steroid withdrawal
25
Q

What are some potential complications of long-term excessive glucocorticoid replacement?

A
  • Secondary Cushing’s Syndrome
  • Osteopenia/Osteoporosis
  • Hypertension w/ hypokalaemia

Adjust doses to manage these complications

26
Q

Why is sudden cessation of steroid treatment not recommended?

A

Can cause adrenal crisis