Adrenal insufficiency

Question 1

outline the typical presentation of adrenal insufficiency?

Answer 1

Increased pigmentation, postural hypotension, fatigue, weakness, myalgia, D+V

Question 2

define adrenal insufficiency?

Answer 2

Deficiency of adrenal cortical hormones (e.g. mineralocorticoids, glucocorticoids and androgens)

Question 3

outline the aetiology

Answer 3

Primary Adrenal Insufficiency

• Addison’s disease(usually autoimmune or TB)

Secondary Adrenal Insufficiency

• Pituitary or hypothalamic disease(could be caused by steroids as will suppress ACTH and cause atrophy)
• ACTH would be low in this situation

Infections

• Tuberculosis
• Meningococcal septicaemia (Waterhouse-Friderichsen Syndrome)
• Adrenal hemorrhage=> abdo pain, fatigue, weakness, dizziness, V
• 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 4

what are the presenting symptoms of adrenal insufficiency ?

Answer 4

• Dizziness
• Anorexia
• Weight loss
• Diarrhoea and Vomiting
• Abdominal pain
• Lethargy
• Weakness
• Depression

Question 5

outline the acute presentation of adrenal insufficiency?

Answer 5

ADDISONIAN CRISIS

Acute adrenal insufficiency

Major haemodynamic collapse: shock, N+V, hypothermia and abdo pain

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

Question 6

what are the signs of adrenal insufficiency on physical examination ?

Answer 6

• 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)

Addisonian Crisis Signs

• Hypotensive shock
• Tachycardia
• Pale
• Cold
• Clammy
• Oliguria

Question 7

what are the investigations done in an addisonian crisis?

Answer 7

FBC (neutrophilia –> infection)

U&Es

• High urea
• Low sodium
• High potassium
• High calcium

CRP/ESR

Glucose - low

Blood cultures

Urinalysis

Culture and sensitivity

Question 8

what are the other tests used to find the cause of adrenal insufficinecy

Answer 8

Autoantibodies (against 21-hydroxylase)

Abdominal CT or MRI

Other tests (adrenal biopsy, culture, PCR)

Question 9

what are the tests that can be used to confirm the diagnosis of adrenal insufficiency?

Answer 9

9 am Serum Cortisol (< 100 nmol/L is diagnostic of adrenal insufficiency)

Random cortisol > 550 nmol/L makes adrenal insufficiency unlikely

If ACTH levels are raised, it indicates primary insufficiency vs low levels indicating secondary insufficiency

Question 10

describe the short synacthen and long synacthen tests for adrenal insufficiency?

Answer 10

Short Synacthen Test

• Give IM 250 µg tetrocosactrin (synthetic ACTH)
• Serum cortisol < 550 nmol/L at 30 mins indicates adrenal failure
• Identify the level of the defect in the hypothalamo-pituitary-adrenal axis

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 11

describe the management for an addisonian crisis?

Answer 11

• Rapid IV fluid rehydration
• 50 mL of 5% dextrose to correct hypoglycaemia
• IV or Im 100 mg hydrocortisone bolus
• Followed by 100 mg 6 hourly hydrocortisone until BP is stable
• Treat precipitating cause (e.g. antibiotics for infection)
• Monitor

Question 12

describe the management for chronic adrenal insufficiency?

Answer 12

repacement of:

Glucocorticoids with hydrocortisone (3/day)

Mineralocorticoids with fludrocortisone

Hydrocortisone dosage needs to be increased during times of acute illness or stress

NOTE: if the patient also has hypothyroidism, give hydrocortisone BEFORE thyroxine (to prevent precipitating an Addisonian crisis)

Question 13

what advice should be given to patients with adrenal insufficiency?

Answer 13

Have a steroid warning card

Wear a medic-alert bracelet

Emergency hydrocortisone on hand

Question 14

what are the complications of adrenal insufficiency?

Answer 14

HYPERKALAEMIA– due to hypoaldosteronism (aldosterone is important in potassium excretion)

Death during Addisonian crisis

Osteopenia/osteoporosis

Question 16

summarise the prognosis of patients with adrenal insufficiency?

Answer 16

Adrenal function rarely recovers

Normal life expectancy if treated

autoimmune polyendocrine syndrome

Question 17

what are the 2 types of autoimmune polyendocrine sydrome and what are the conditions included in each?

Answer 17

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 18

outline the epidemiology of adrenal insufficiency?

Answer 18

Most common cause is IATROGENIC

Primary causes are rare

Question 19

what are the risk factors for adrenal insufficiency?

Answer 19

• female sex
• adrenocortical autoantibodies
• adrenal haemorrhage
• autoimmune diseases