Adrenal Insufficiency

Question 1

What is Adrenal Insufficiency?

Answer 1

Deficiency of adrenal cortical hormones

Question 2

What are some examples of adrenal cortical hormones?

Answer 2

Mineralocorticoids

Glucocorticoids

Androgens

Question 3

What are some of the causes and risk factors of adrenal insufficiency?

Answer 3

Primary Adrenal Insufficiency

Secondary Adrenal Insufficiency

Infections

Infiltration

Infarction

Inherited

Surgical
Iatrogenic

Question 4

What are some examples of a Primary Adrenal Insufficiency?

Answer 4

Addison’s Disease (usually autoimmune)

Question 5

What are some examples of Secondary Adrenal Insufficiency?

Answer 5

Pituitary or Hypothalamic Disease

Question 6

What are some examples of infections?

Answer 6

Tuberculosis

Meningococcal septicaemia (Waterhouse-Friderichsen Syndrome)
CMV
Histoplasmosis

Question 7

What are some examples of Infiltration?

Answer 7

Metastasis (mainly from lung, breast, melanoma)

Lymphomas

Amyloidosis

Question 8

What is an example of an Infarction cause of adrenal insufficiency?

Answer 8

Secondary to thrombophilia

Question 9

What is an inherited cause of Adrenal Insuffiency?

Answer 9

Adrenoleukodystrophy

ACTH receptor mutation

Question 10

What is a surgical cause of Adrenal Insuffiency?

Answer 10

After bilateral adrenalectomy

Question 11

What is the Iatrogenic cause of adrenal insuffiency?

Answer 11

Sudden cessation of long term steroid therapy

Question 12

What is the epidemiology of adrenal insufficiency?

Answer 12

Most common cause is IATROGENIC

Primary causes are rare

Question 13

What is the chronic presentation of adrenal insufficiency?

Answer 13

Dizziness

Anorexia

Weight Loss

Diarrhoea and Vomiting

Abdominal Pain

Lethargy

Weakness

Depression

Question 14

What is the acute presentation of adrenal insufficiency (Addisonian Crisis)?

Answer 14

Acute Adrenal Insufficiency

Major Haemodynamic Collapse

Precipitated by stress (e.g. infection, surgery)

Question 15

What are the signs of adrenal insufficiency on physical examination?

Answer 15

• Postural Hypotension
• Increased pigmentation
  
  • More noticeable on buccal mucosa, scars, skin creases, nails and pressure points
• Loss of body hair in women (due to androgen deficiency)
• Associated autoimmune condition (e.g. vitiligo)

Question 16

What are the signs of Addisonian Crisis?

Answer 16

Hypotensive Shock

Tachycardia

Pale

Cold
Clammy

Oliguria

Question 17

What are some of the investigations for adrenal insufficiency?

Answer 17

To confirm the diagnosis

Identify the level of the defect in the hypothalamo-pituitary-adrenal axis

Identify the Cause

Check TFTs

Question 18

How do you confirm the diagnosis for adrenal insufficiency?

Answer 18

• 9 am Serum Cortisol (< 100 nmol/L is diagnostic of adrenal insufficiency)
  
  • >550 nmol/L makes adrenal insufficiency unlikely
• Short Synacthen Test
  
  • IM 250 µg tetrocosactrin (synthetic ACTH)
  • Serum cortisol < 550nmol/L at 30 mins indicates adrenal failure

Question 19

How do you identify the level of the defect in the hypothalamo-pituitary-adrenal axis?

Answer 19

• HIGH in primary disease
• LOW in secondary
• LONG SYNACTHEN TEST
  
  • 1 mg synthetic ACTH administered
  • Measure serum cortisol at 0, 30, 60, 90 and 120 minutes
  • Then measure again at 4, 6, 8, 12 and 24 hours
  • Patients with primary adrenal insufficiency show no increased after 6 hours

Question 20

How do you identify the cause of adrenal insufficiency?

Answer 20

Autoantibodies (against 21-hydroxylase)

Abdominal CT or MRI

Other tests (adrenal biopsy, culture, PCR)

Question 21

What investigations would you do for Addisoninan Crisis?

Answer 21

• FBC (neutrophilia –> infection)
• U&Es
  
  • High Urea
  • Low Sodium
  • High Potassium
• CRP/ESR
• Calcium (may be raised)
• Glucose - low
• Blood Cultures
• Urinalysis
• Culture and Sensitivity

Question 22

What is the management plan for adrenal insufficiency?

Answer 22

Rapid IV fluid rehydration

50 mL of 50% dextrose to correct hypoglycaemia

IV 200 mg hydrocortisone bolus

Followed by 100 mg 6 hourly hydrocortisone until BP is stable

Treat precipitating cause (e.g. antibiotics for infection)

Monitor

Question 23

What is the management for Chronic Adrenal Insufficiency?

Answer 23

• Replacement of:
  
  • Glucocorticoids with hydrocortisone (3/day)
  • Mineralocorticoids with fludrocortisone
• Hydrocortisone dosage needs to be increased during times of acute illness or stress
• If the patient also has hypothyroidism, give hydrocortisone BEFORE thyroxine

Question 24

What advice can you get for adrenal insufficiency?

Answer 24

Have a steroid warning card

Wear a medic-alert bracelet

Emergency Hydrocortisone on hand

Question 25

What are the possible complications of adrenal insufficiency?

Answer 25

Hyperkalaemia
Death during Addisonian Crisis

Question 26

What is the prognosis for patients with adrenal insufficiency?

Answer 26

Adrenal function rarely recovers

Normal life expectancy if treated

Question 27

What is Autoimmune Polyendocrine Syndrome?

Answer 27

Type 1 - autosomal recessive disorder caused by mutations in the AIRE gene

Consists of the following diseases:

• Addison’s Disease
• Chronic Mucocutaneous Candidiasis
• Hypoparathyroidism

Type 2 - also known as Schmidt’s Syndrome

• Addison’s Disease
• Type 1 Diabetes
• Hypothyroidism
• Hypogonadism