Addison's disease Flashcards
What is Addison’s disease?
Adrenal insufficiency. Reduced amounts of cortisol and aldosterone being produced.
What is the pathophysiology of Addison’s Disease?
Decreased production of adrenocortical hormones due to either destruction of the adrenal cortex or disruption of hormone synthesis. Infiltrating diseases (e.g. TB or metastasis) can also cause destruction of the adrenal medulla.
What are the 3 layers of the adrenal cortex?
Zona Glomerulosa
Zona Fasciculata
Zona Reticularis
What are the 3 adrenocortical hormones?
- Aldosterone
- Cortisol
- Dehydroepiandrosterone
What is primary adrenal insufficiency?
Reduced secretion of adrenal hormones due to destruction or dysfunction of the adrenal gland
What is secondary adrenal insufficiency?
Inadequate pituitary adrenocorticotrophic hormone (ACTH) release and subsequent cortisol production.
What is the most common cause of primary hypoadrenalism in the UK?
Autoimmune destruction of the adrenal glands (80% of cases)
What are other primary causes of Addison’s disease?
Primary causes
- TB (most prevalent cause in endemic countries)
- Metastases (e.g. bronchial carcinoma)
- Meningococcal septicaemia (Waterhouse-Friderichsen syndrome)
- HIV
- Antiphospholipid syndrome
What are secondary causes of Addison’s disease?
Secondary causes
- Pituitary disorders (e.g. tumours, irradiation, infiltration)
- Exogenous glucocorticoid therapy
What are symptoms of Addison’s disease?
- Lethargy / Weakness
- Anorexia / weight loss
- Dizziness (less common)
- Arthralgia / Myalgia (less common)
- GI symptoms
- Nausea & vomiting
- Abdo pain
- Constipation
- Salt-craving (Hyponatraemia / Hyperkalaemia)
History should be directed at establishing the underlying cause, such as a known history of autoimmune disease, HIV infection, possible tuberculosis infection, and current medication use.
Sudden alterations in glycaemic control and recurrent hypoglycaemia in patients with type 1 diabetes mellitus may suggest autoimmune polyglandular syndrome type 2 with co-existing diabetes and Addison’s disease
What are the signs of Addison’s disease found on examination?
- Mucosal & cutaneous hyperpigmentation
- Of palmar creases, knuckles, scars & buccal mucosa
- Hypotension (Postural)
- Signs of autoimmunity
- Vitiligo
- Hashimoto’s thyroiditis
- Pernicious anaemia
- Loss of axillary and pubic hair
How can you distinguish primary adrenal insuffiency from secondary adrenal insufficiency from the presentation alone?
Primary adrenal insufficiency is associated with hyperpigmentation whereas secondary adrenal insufficiency is not.
What investigations are used to confirm a diagnosis of Addison’s disease?
1. Routine bloods (FBC, U&Es)
- Don’t provide diagnosis but can be useful to confirm presence of anaema, hyperkalaemia, hyponatraemia, etc.
2. 9am serum cortisol
+ve result = LOW
- Used in primary care or when Synacthen test unavailable
3. ACTH stimulation (Synacthen) test = Definitive investigation
4. Additional tests
- ACTH test
- Autoantibody test
- Insulin hypoglycaemia test
How would you interpret the results of a 9am Serum Cortisol Test?
- > 500 nmol/l = Normal - Addison’s very unlikely
- 100-500 nmol/l = ACTH stimulation test should be performed
- < 100 nmol/l = Abnormal
You perform U&E’s and VBG on a patient with Addison’s disease. What abnormalities may you find?
- Hyperkalaemia
- Hyponatraemia
- Metabolic acidosis
- Hyperglycaemia