Addisons disease

Question 1

What is Addison’s disease?

Answer 1

Addison’s disease is the autoimmune destruction of the adrenal glands, leading to primary hypoadrenalism, which accounts for 80% of cases in the UK.

Question 2

What hormones are reduced in Addison’s disease?

Answer 2

Cortisol and aldosterone are reduced in Addison’s disease.

Question 3

What are common features of Addison’s disease?

Answer 3

Common features include lethargy, weakness, anorexia, nausea & vomiting, weight loss, and ‘salt-craving’.

Question 4

What skin changes are associated with Addison’s disease?

Answer 4

Hyperpigmentation, especially in palmar creases, is associated with Addison’s disease.

Question 5

What is the relationship between ACTH and hyperpigmentation?

Answer 5

ACTH is derived from proopiomelanocortin (POMC); its cleavage also produces melanocyte-stimulating hormones (MSH), which stimulate melanin production, leading to hyperpigmentation.

Question 6

How does primary Addison’s disease differ from secondary adrenal insufficiency?

Answer 6

Primary Addison’s disease is associated with hyperpigmentation, whereas secondary adrenal insufficiency is not.

Question 7

What are some additional features of Addison’s disease?

Answer 7

Additional features may include vitiligo, loss of pubic hair in women, hypotension, hypoglycaemia, hyponatraemia, and hyperkalaemia.

Question 8

What are the symptoms of an Addisonian crisis?

Answer 8

Symptoms of an Addisonian crisis include collapse, shock, and pyrexia.

Question 9

What are primary causes of hypoadrenalism?

Answer 9

Primary causes include tuberculosis, metastases (e.g. bronchial carcinoma), meningococcal septicaemia (Waterhouse-Friderichsen syndrome), HIV, and antiphospholipid syndrome.

Question 10

What are secondary causes of hypoadrenalism?

Answer 10

Secondary causes include pituitary disorders such as tumours, irradiation, and infiltration.

Question 11

What is a common treatment-related cause of hypoadrenalism?

Answer 11

Exogenous glucocorticoid therapy can lead to hypoadrenalism.

Question 12

What is the definite investigation for suspected Addison’s disease?

Answer 12

The ACTH stimulation test (short Synacthen test).

Question 13

What is measured during the ACTH stimulation test?

Answer 13

Plasma cortisol is measured before and 30 minutes after giving Synacthen 250ug IM.

Question 14

What adrenal autoantibodies may be demonstrated in Addison’s disease?

Answer 14

Adrenal autoantibodies such as anti-21-hydroxylase.

Question 15

What can be useful if an ACTH stimulation test is not readily available?

Answer 15

Sending a 9 am serum cortisol.

Question 16

What does a serum cortisol level > 500 nmol/l indicate?

Answer 16

It makes Addison’s very unlikely.

Question 17

What does a serum cortisol level < 100 nmol/l indicate?

Answer 17

It is definitely abnormal.

Question 18

What should be done if serum cortisol is between 100-500 nmol/l?

Answer 18

An ACTH stimulation test should be performed.

Question 19

What electrolyte abnormalities are associated with undiagnosed Addison’s disease?

Answer 19

Hyperkalaemia, hyponatraemia, hypoglycaemia, and metabolic acidosis.

Question 20

What is the primary treatment for Addison’s disease?

Answer 20

Patients with Addison’s disease are usually given both glucocorticoid and mineralocorticoid replacement therapy.

Question 21

What is the common glucocorticoid used in Addison’s disease management?

Answer 21

Hydrocortisone is commonly used, typically in 2 or 3 divided doses.

Question 22

What is the usual daily dosage of hydrocortisone for Addison’s disease?

Answer 22

Patients typically require 20-30 mg per day, with the majority given in the first half of the day.

Question 23

What mineralocorticoid is used in Addison’s disease management?

Answer 23

Fludrocortisone is used as part of the treatment.

Question 24

What is an important aspect of patient education for Addison’s disease?

Answer 24

Emphasise the importance of not missing glucocorticoid doses.

Question 25

What should patients consider for emergencies related to Addison’s disease?

Answer 25

Consider MedicAlert bracelets and steroid cards.

Question 26

What should patients be provided with for an adrenal crisis?

Answer 26

Patients should be provided with hydrocortisone for injection with needles and syringes.

Question 27

How should glucocorticoid doses be adjusted during an intercurrent illness?

Answer 27

The glucocorticoid dose should be doubled, while the fludrocortisone dose stays the same.

Question 28

Where can guidelines for managing intercurrent illness in Addison’s disease be found?

Answer 28

The Addison’s Clinical Advisory Panel have produced guidelines detailing particular scenarios.

Question 29

What are the causes of Addisonian crisis?

Answer 29

Sepsis or surgery causing an acute exacerbation of chronic insufficiency (Addison’s, Hypopituitarism), adrenal haemorrhage (e.g., Waterhouse-Friderichsen syndrome), and steroid withdrawal.

Question 30

What is the management for Addisonian crisis?

Answer 30

Hydrocortisone 100 mg IM or IV, 1 litre normal saline infused over 30-60 mins or with dextrose if hypoglycaemic. Continue hydrocortisone 6 hourly until the patient is stable.

Question 31

Is fludrocortisone required during Addisonian crisis management?

Answer 31

No, fludrocortisone is not required because high cortisol exerts weak mineralocorticoid action.

Question 32

When can oral replacement begin after an Addisonian crisis?

Answer 32

Oral replacement may begin after 24 hours and be reduced to maintenance over 3-4 days.