06.02 Endo/Metabolic
06.02 Adrenals [PDF link]: Cushing's disease & syndrome – cortisol. Adrenal insufficiency – Addison’s disease, adrenal crisis. Hyperaldosteronism – Conn’s syndrome. Phaeochromocytoma.
External links
NICE guidelines on recognition and treatment of adrenal insufficiency: NICE guidelines
BMJ article summarising NICE guidelines + nice infographic: BMJ
More on Cushing’s
NICE CKS page on Cushing’s syndrome, last updated Dec 2024
The page on investigations is quite useful.
More regarding adrenal insufficiency
In patients with suspected adrenal sufficiency, do early morning cortisol (between 8-9am) – low cortisol should be seen in adrenal insufficiency
<150 nmol/L is considered low
150-300 is borderline
>300 – unlikely to have adrenal insufficiency
Needs to be early morning cortisol – random cortisol levels are not as helpful as they can be normally low. Cortisol varies diurnally, with higher levels in the early morning. If early morning cortisol levels are low (when they are meant to be high) this is more reliably interpretable. [See also NICE CKS for more info.]
Borderline levels will benefit from repeating
In adrenal insufficiency where patients are unwell, they need to be started on steroids asap.
Hydrocortisone total daily dose 15 mg to 25 mg orally in 2 to 4 divided doses, which can be split by giving a larger dose in the morning. Prednisolone can also be given.
Fludrocortisone is only given in patients with primary adrenal insufficiency.
In adrenal crisis, steroid doses must be increased
Hydrocortisone ≥40 mg in 2-4 divided doses (or prednisolone equivalent – ≥10 mg in 1-2 divided doses) until acute phase of illness over
Patients already on ≥10 mg daily do not require increased dosing, but can split their doses into 2 doses throughout the day
If unable to take orally (eg diarrhoea/vomiting), give hydrocortisone 100 mg IM/IV
Consider giving emergency management kit with IM hydrocortisone that can be administered by patients or family members.
Autoimmune polyendocrine syndrome (APS; aka autoimmune hypoadrenalism)
Review article – Husebye ES, Anderson MS, Kämpe O. Autoimmune Polyendocrine Syndromes. New England Journal of Medicine 2018. https://doi.org/10.1056/NEJMra1713301.
D: Group of conditions characterised by functional impairment of multiple endocrine glands secondary to autoimmunity.
Generally ÷ type 1 and type 2.
APS type 1 – autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)
Autosomal recessive, due to mutation in autoimmune regulator gene on chromosome 21
Diagnostic criteria – 2 of 3 cardinal components during childhood:
Chronic mucocutaneous candidiasis
Hypoparathyroidism
Primary adrenal insufficiency (Addison’s)
Other s/smx:
Enamel hypoplasia
GI smx: chronic diarrhea or constipation
Primary ovarian insufficiency
Vitiligo
Eyes: b/l keratitis, photophobia
Periodic fever with rash
Autoimmune-induced hepatitis, pneumonitis, nephritis, pancreatitis and asplenia
APS type 2
Some HLA related associations with different autoimmune diseases – HLA DR3/DR4.
Diagnostic criteria – 2 of 3 endocrinopathies:
T1DM
Autoimmune thyroid disease
Primary adrenal insufficiency (Addison’s)
