Adrenal insufficiency

Question 1

Define adrenal insufficiency

Answer 1

Deficiency of adrenal cortical hormones

e.g. mineralocorticoids, glucocorticoids, androgens

Question 2

Aetiology of adrenal insufficiency

8 - 1,1,4,3,1,2,1,1

Answer 2

Primary adrenal insufficiency
Addison’s disease (usually autoimmune)

Secondary adrenal insufficiency
Pituitary or hypothalamic disease

Infections
TB
Meningococcal septicaemia (Waterhouse-Friderichsen syndrome)
CMV
Histoplasmosis 

Infiltration
Metastasis (mainly from lung, breast, melanoma)
Lymphomas
Amyloidosis

Infarction
Secondary to thrombophilia

Inherited
Adrenoleukodystrophy
ACTH receptor mutation

Surgical
After bilateral adrenalectomy

Iatrogenic
Sudden cessation of long term steroid therapy

Question 3

Epidemiology of adrenal insufficient

causes x2

Answer 3

Most common cause is IATROGENIC

Primary causes are rare

Question 4

Presenting symptoms of adrenal insufficiency

2 groups

Answer 4

Chronic presentation

Acute presentation - Addisonian crisis

Question 5

Presenting symptoms of adrenal insufficiency - chronic

9

Answer 5

(symptoms tend to be vague & non specific)
Dizziness
Anorexia
Weight loss
Diarrhoea & vomiting
Abdominal pain
Lethargy 
Weakness
Depression

Question 6

Presenting symptoms of adrenal insufficiency - acute/addisonian crisis
(3)

Answer 6

Acute adrenal insufficiency
Major haemodynamic collapse
Precipitated by stress (e.g. infection, surgery)

Question 7

Signs of adrenal insufficiency on physical examination

2 groups

Answer 7

Adrenal insufficiency

Addisonian crisis

Question 8

Signs of adrenal insufficiency on physical examination - adrenal insufficiency
(4)

Answer 8

Postural hypotension
Increased pigmentation
Loss of body hair in women (due to androgen deficiency)
Associated autoimmune condition (e.g. vitiligo)

Question 9

Signs of adrenal insufficiency on physical examination - addisonian crisis
(6)

Answer 9

Hypotensive shock
Tachycardia
Pale
Cold
Clammy
Oliguria

Question 10

Investigations for adrenal insufficiency

5 groups

Answer 10

To confirm diagnosis
Identify level of defect in hypothalamo-pituitary-adrenal axis
Identify cause 
TFTs
Investigations in Addisonian crisis

Question 11

Investigations for adrenal insufficiency - to confirm diagnosis
(2)

Answer 11

9am serum cortisol
<100nmol/L is diagnostic of adrenal insufficiency
>550nmol/L makes it unlikely
Short synacthen test
IM 250microg tetrocosactrin (synthetic ACTH)
serum cortisol <550nmol/L at 30mins indicates adrenal failure

Question 12

Investigations for adrenal insufficiency - identify level of defect in hypothalamo-pituitary-adrenal axis
(3)

Answer 12

HIGH in primary disease
LOW in secondary disease
Long synacthen test
1mg synthetic ACTH administered
measure serum cortisol at 0, 30, 60, 90, 120 mins
measure agin at 4, 6, 8, 12, 24 hrs
patients w/ primary adrenal insufficiency have no increase after 6 hrs

Question 13

Investigations for adrenal insufficiency - identify cause

3

Answer 13

Autoantibodies (against 21-hydroxylase)
Abdo CT/MRI
Other tests (adrenal biopsy, culture, PCR)

Question 14

Investigations for adrenal insufficiency - addisonian crisis

8

Answer 14

FBC (neutrophilia = infection)
U&Es
high urea & potassium
low sodium
CRP/ESR
Calcium (may be raised)
Glucose (low)
Blood cultures
Urinalysis
Culture & sensitivity

Question 15

Management of adrenal insufficiency

3 groups

Answer 15

Addisonian crisis
Chronic adrenal insufficiency
Advice

Question 16

Management of adrenal insufficiency - addisonian crisis

6

Answer 16

Rapid IV fluid rehydration
50ml 50% dextrose to correct hypoglycaemia
IV 200mg hydrocortisone bolus
Followed by 100mg 6 hrly hydrocortisone until BP stable
Treat precipitating cause (e.g. infection)
Monitor

Question 17

Management of adrenal insufficiency - chronic adrenal insufficiency
(3)

Answer 17

Replacement of glucocorticoids w/ hydrocortisone (x3 daily)
Replacement of mineralocorticoids w/ fludrocortisone
Hydrocortisone dosage needs to be increased in acute illness or stress

Question 18

Management of adrenal insufficiency - advice

3

Answer 18

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

Question 19

Complications of adrenal insufficiency

2

Answer 19

HYPERKALAEMIA

Death during Addisonian crisis

Question 20

Prognosis of adrenal insufficiency

3

Answer 20

Adrenal function rarely recovers
Normal life expectancy if treated

Autoimmune polyendocrine syndrome

Question 21

Autoimmune polyendocrine syndrome type 1 & 2

3 + 4

Answer 21

TYPE 1 = autosomal recessive disorder caused by mutations in AIRE gene. Consists of:
Addison’s disease
Chronic mucocutaneous candidiasis
Hypoparathyroidism

TYPE 2 (aka Schmidt’s syndrome)
Addison’s disease
T1DM
Hypothyroidism 
Hypogonadism