Adrenal insufficiency

Question 1

What does adrenal insufficiency refer to

Answer 1

A reduced production of the hormones secreted by the adrenal cortex

Question 2

Causes of primary adrenal insufficiency include

Answer 2

Diseases affecting the adrenal cortex such as Addison’s disease

Question 3

Causes of secondary adrenal insufficiency include

Answer 3

Pituitary/ hypothalamic disease or long term steroid use and suppression of the hypothalamic pituitary adrenal (HPA) axis

Question 4

What is Addison’s disease

Answer 4

an isolated autoimmune adrenal insufficiency which occurs predominantly in males and females in the 3rd decade
Thereafter, predominantly females

Question 5

What is the most common cause of primary adrenal insufficiency

Answer 5

autoimmune adrenalitis

Question 6

What is the first evidence of autoimmune adrenal insufficiency

Answer 6

an increase in plasma renin activity, suggesting that the son glomerulosa failure and reduction in aldosterone occur first

Question 7

What causes APS (Autoimmune adrenal insufficiency) type 1

Answer 7

Autosomal recessive disorder caused by mutations in the AIRE gene which encodes a nuclear transcription factor

Question 8

What is characteristic of APS type 1

Answer 8

chronic mucocutaneous candidiasis and hypoparathyroidism followed by Addisons

Question 9

What is APS type 2

Answer 9

an autosomal recessive, dominant or polygenic manner that is characterised by Addison’s disease, autoimmune thyroid disease and diabetes mellitus

Question 10

What are some of the causes of primary adrenal insufficiency

Answer 10

Autoimmune
Infection - TB, fungal, cytomegalovirus
Infiltration - metastases, lymphoma, amyloidosis, haemochromatosis
Infarction - due to thrombosis caused by thrombophilia
Haemorrhage - meningococcal septicaemia , anticoagulants

Question 11

What is the second most common cause of Addison’s disease

Answer 11

TB

Question 12

Name the groups of patients that are likely to have adrenal insufficiency secondary to HPA axis suppression by longterm glucocorticoid use

Answer 12

Those who have received a glucocorticoid dose equivalent to or more than 20mg of prednisolone per day for more than 3 weeks
those who have received an evening or bedtime dose of prednisone or more than a few weeks
those who have a Cushingoid appearance

Question 13

How does adrenal iris most commonly present as

Answer 13

Shock

Question 14

What patients might an acute adrenal crisis be seen in

Answer 14

those with previously undiagnosed adrenal insufficiency who have been subject to acute stress or illness (e.g. infection)
those with known adrenal insufficiency who have not increased their steroid does during and infection or other illness, or who have been vomiting

Question 15

Describe the clinical presentation of primary adrenal insufficiency

Answer 15

Malaise, fatigue, weakness, anorexia, weight loss
Nausea, vomiting, abdo pain, diarrhoea
postural hypotension, improved BP in hypertensive patients
Hyponatraemia, hyperkalaemia, hypoglycaemia, hypercalcaemia
Hyperpigmentation
Impairment of memory, confusion, depression

Question 16

What can be given to cause the hyerpigmentation to disappear

Answer 16

A few months of treatment with glucocorticoids

Question 17

What do vitiligo resit from

Answer 17

autoimmune destruction of dermal melanocytes

Question 18

What are some of the differences in the presenting complaint of secondary and primary adrenal insufficiency

Answer 18

Secondary - no hyperpigmentation
No dehydration or hyperkalaemia
GI symptoms are less common
Hypoglycaemia is more common

Question 19

What would be seen in a blood test at presentation

Answer 19

Hyponatraemia 
Hyperkalaemia 
Acidosis 
High urea 
Mild hypercalcaemia 
eosinophilia 
Hypoglycaemia (rare in adults)

Question 20

When should cortisol be measured

Answer 20

9am

Question 21

What level of cortisol is diagnostic of adrenal insufficiency

Answer 21

9 am serum cortisol level of less than 100nmol/L

Question 22

What is the next step for those who have a 8-9am cortisol level of 100-550nmol/L

Answer 22

short ACTH stimulation test –> 250g of synthetic ACTH is given intramuscularly and serum cortisol is measured at time 0, 30 mins and 60 mins

Question 23

What is a normal response to ACTH stimulation test

Answer 23

Peak cortisol of over 550nmol/L

Question 24

What sort of test may be used to distinguish primary from secondary adrenal insufficiency

Answer 24

Prolonged ACTH stimulation test

Question 25

What other conditions should patients with autoimmune adrenal failure also be investigated for

Answer 25

Diabetes mellitus: fasting glucose
Thyroid disease: Free T3/T4 and TSH
parathyroid dysfunction: calcium and phosphate
Pernicious anaemia: parietal cell antibodies
Primary gonadal failure: LH, FSH, either testosterone or estradiol

Question 26

What are the investigations required in an Addisonian crisis

Answer 26

Blood for serum cortisol ACTH, renin and serum urea and electrolytes should be drawn and therapy started immediately

Question 27

What are the 3 main treatments initially for an Addisonian crisis

Answer 27

Fluids - 1-3L of saline IV with IV dextrose if hypoglycaemic
Glucocorticoids: dexamethasone IV
Underlying cause - infection etc.

Question 28

Why is dexamethasone preferred to hydrocortisone

Answer 28

Not measured by serum cortisol assays

Question 29

Describe the long term treatment for adrenal insufficiency

Answer 29

Glucocorticoid replacement: hydrocortisone - 1-mg am, 5mg noon and 5mg evening
doubling this dose at times of intercurrent illness

Emergency supply of IM hydrocortisone - times of vomiting on their way to hospital

steroid card and medic alert bracelet

Question 30

What other replacements may be required for patients with adrenal insufficiency

Answer 30

Mineralocorticoid replacement (fludrocortisone) 
Androgen replacement

Question 31

What should we do for patients who have a withdrawal from glucocorticoids

Answer 31

The dose must be reduced gradually