Adrenal insufficiency

Question 1

What is adrenal insufficiency?

Answer 1

deficiency of adrenal cortical hormones (e.g. mineralocorticoids, glucocorticoids + androgens)

Question 2

What is primary adrenal insufficiency? What is the most common cause in the UK? How about worldwide?

Answer 2

Addison’s disease
UK: AI
Worldwide: TB

Question 3

What can cause secondary adrenal insufficiency?

Answer 3

Inadequate ACTH from Pituitary
Infiltration: Mets (lung), Lymphomas, Amyloidosis
Iatrogenic: Sudden cessation of long-term steroid therapy

Question 4

Describe the epidemiology of Addisons disease

Answer 4

Rare
UK prevalence: 8,400
Often 30-50 yrs
F > M

Question 5

List 6 symptoms seen in Addisons

Answer 5

Dizziness 
Weight loss + Anorexia   
D+V
Abdominal pain 
Lethargy + Weakness  
Depression

Question 6

How does Addisons present?

Answer 6

Acute: crisis precipitated by infection, surgery or trauma: hypotension, hypovolaemic shock, acute abdominal pain, low-grade fever + vomiting.
Chronic: symptoms develop insidiously, may be mild + non specific

Question 7

List 4 signs of adrenal insufficiency

Answer 7

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

Question 8

How is adrenal insufficiency confirmed?

Answer 8

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

Question 9

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

Answer 9

ACTH HIGH in primary disease
ACTH LOW in secondary

Long Synacthen Test
1 mg synthetic ACTH administered
Measure serum cortisol at 0, 30, 60, 90 + 120 minutes
Measure again at 4, 6, 8, 12 + 24 hours
Primary: no increase after 6 hours
Secondary: Delayed normal response

Question 10

How do you identify the cause of adrenal insufficiency?

Answer 10

Autoantibodies (against 21-hydroxylase)
Abdo CT or MRI: exclude infection, haemorrhage, or mets
CXR: exclude lung malignancy
TFTs: often have hypothyroidism

Question 11

What investigations are performed in an addisonian crisis?

Answer 11

FBC: neutrophilia = infection
U+Es: High urea, Low Na+, High K+
CRP (High in acute infection)
Calcium (may be raised) 
Glucose LOW
Blood cultures  
Urinalysis: MC+S  (UTI may have triggered crisis)
CXR: identify cause e.g. TB, carcinoma or precipitant e.g. infection

Question 12

How is an addisonian crisis treated?

Answer 12

Rapid IV fluid rehydration
50 mL of 50% dextrose to correct hypoglycaemia
Calcium glucunate if appropriate
IV 100 mg HYDROCORTISONE bolus
Repeated 6 hourly hydrocortisone until BP is stable
Treat precipitating cause (e.g. abx for infection)
Monitor

Question 13

How is chronic adrenal insufficiency treated?

Answer 13

Hydrocortisone 10/5/5mg or Prednisolone 3-4mg OD
Fludrocortisone 50-100mcg OD
Hydrocortisone dosage needs to be increased during times of acute illness or stress

Question 14

What advice is given to patients with adrenal insufficiency?

Answer 14

Have a steroid warning card
Wear a medic-alert bracelet
Emphasise importance of not missing doses
Provide emergency hydrocortisone (IM) for injection with needles + syringes for adrenal crisis/ too unwell for PO meds

Question 15

In simple term, describe management of adrenal insufficiency with intercurrent illness

Answer 15

Glucocorticoid dose should be doubled
Fludrocortisone dose stays the same

Question 16

Give 2 complications of adrenal insufficiency

Answer 16

HYPERKALAEMIA
Death during Addisonian crisis

Question 17

What is the prognosis in adrenal insufficiency?

Answer 17

Adrenal function rarely recovers
Normal life expectancy if treated

Question 18

What is type 1 AI polyendocrine syndrome?

Answer 18

Autosomal recessive disorder caused by mutations in the AIRE gene. Consists of the following diseases:
Addison’s disease
Chronic mucocutaneous candidiasis
Hypoparathyroidism

Question 19

What is type 2 AI polyendocrine syndrome?

Answer 19

AKA: Schmidt's Syndrome 
Addison's disease  
T1DM  
Hypothyroidism 
Hypogonadism

Question 20

What 6 signs may be seen in an addisonian crisis?

Answer 20

Hypotensive shock 
Tachycardia 
Pale  
Cold  
Clammy  
Oliguria

Question 21

How do you administer drugs for patients with concurrent hypothyroidism and adrenal insufficiency?

Answer 21

Hydrocortisone BEFORE thyroxine (to prevent precipitating an Addisonian crisis)
Giving thyroxine first can enhance cortisol clearance + precipitate an adrenal crisis (increases BMR leading to increased requirement of cortisol)

Question 22

What bloods are usually seen in Addisons?

Answer 22

High K+
Low Na+
Metabolic acidosis (less H+ excretion)

Question 23

What should be done if Addisonian crisis is suspected whilst awaiting blood results?

Answer 23

DONT wait for results before administering tx.
Take blood for random cortisol + treat immediately if appropriate