Adrenal insufficency

Question 1

Define:

Answer 1

Deficiency of adrenal cortical hormones

Question 2

Aetiology/risk factors:

Answer 2

Primary - Addison’s disease (autoimmune + most common cause in the UK)

Secondary - Hypothalamic/pituitary disease

Iatrogenic - when there is a sudden cessation of long term steroids

Surgical - post bi-lateral adrenalectomy.

Infection - TB (most common cause worldwide), CMV, histoplasmosis, menningcoccal septicaemia

Infarction - secondary to thrombophilia

Inherited - congenital adrenal hyperplasia, ACTH receptor defect

Infiltration - metastasis, amyloidosis and lymphomas

Question 3

Epidemiology:

Answer 3

In the UK 80% is caused by autoimmune

Question 4

Symptoms:

Answer 4

These tend to be vague and non-specific in chronic:

Darkened skin

Lethargy

Depression

Dizziness

Anorexia

Weight loss

Abdo pain

Diarrhoea and vomiting.

Weakness

Question 5

Symptoms of an Addisonian crisis:

Answer 5

Major haemodynamic collapse precipitated by stress e.g. infection

Question 6

Signs:

Answer 6

Darkened skin - especially in buccal mucosa, skin creases, scars and pressure points

Postural hypotension

Other autoimmune conditions such as autoimmune vitiligo

loss of body hair in women due to loss of androgen

Question 7

Signs of an Addisonian crisis:

Answer 7

Tachycardia 
Cold 
Clammy 
Hypotensive shock 
Pale 
Oligouria

Question 8

Investigations:

Answer 8

Low sodium and high potassium

Low glucose due to the lack of cortisol

9am morning cortisol - if this is <100nmol/L as morning should have the highest amount

Short syncacthen test - <550nmol/L of cortisol

ACTH will be high in primary but low in secondary

Autoantibodies e.g. against 21 hydroxylase (CAH)

Abdo CT/MRI

Biopsy, culture and PCR to investigate infections

TFTs

Question 9

Investigations for addisonian crisis:

Answer 9

FBC - neutrophilia = infection 
U+E's 
CRP/ESR
Calcium may be high 
glucose will be low
urinanalysis 
Culture and sensitivity

Question 10

Management of addisonian crisis:

Answer 10

o Rapid IV fluid rehydration
o 50 mL of 50% dextrose to correct hypoglycaemia
o IV 200 mg hydrocortisone bolus
o Followed by 100 mg 6 hourly hydrocortisone until BP is stable
o Treat precipitating cause (e.g. antibiotics for infection)
o Monitor

Question 11

Management:

Answer 11

• Chronic Adrenal Insufficiency
o Replacement of:
• Glucocorticoids with hydrocortisone (3/day)
• Mineralocorticoids with fludrocortisone
o Hydrocortisone dosage needs to be increased during times of acute illness or stress

o Have a steroid warning card
o Wear a medic-alert bracelet
o Emergency hydrocortisone on hand

Question 12

Complications:

Answer 12

Hyperkalaemia

Death during the addisonian crisis

Question 13

Prognosis:

Answer 13

Unlikely to resolve

normal life if adhere to treatment

Autoimmune Polyendocrine Syndrome

Question 14

What are the two types of Autoimmune Polyendocrine Syndrome:

Answer 14

o 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

Question 15

What do the different parts of the adrenal gland produce:

Answer 15

The medulla is made up of Chromaffin cells which produce CATECHOLEAMINES
 Adrenaline 80%
 Noradrenaline 20%
 Dopamine – very small amounts

Zona reticularis produce GLUCOCORTICOIDS (cortisol) and Zona fasiculata produces SEX STEROIDS (androgens and oestrogens).

Zona glomerulosa produces MINERALOCORTICOIDS (aldosterone)