Management and prognosis

Seek specialist advice. (1,2)

Treat cause and replace glucocorticoid and mineralocorticoid, supplemented by careful and persistent education. Patients should carry a steroid card and MedicAlert bracelet.

Primary insufficiency:

• hydrocortisone - 15-25 mg/day, dosage depends on the bodyweight, metabolism and absorption, for example:
  • 10 mg in the morning
  • 5 mg at midday
  • 5 mg in the evening
    
    
• fludrocortisone - in a single dose of 50-200 μg/day, dosage depends on metabolism and exercise levels
  • measure blood pressure and serum electrolytes to assess adequacy of mineralocorticoid therapy
  • complications include hypokalaemia, hypertension, oedema and cardiac enlargement
• adjust doses of both drugs according to postural hypotension, plasma urea and electrolytes
• dehydroepiandrosterone (DHEA) - 25-50 mg/day usually as a single morning dose

Secondary insufficiency:

• as above but may not require mineralocorticoid
• may require thyroxine if there is hypothalamic-pituitary disease

Note Addison's disease may be associated with other autoimmune disease - screening for thyroid disease (autoimmune thyroid disease is associated with Addison's disease) should be undertaken, especially if there is an inadequate response to treatment.

Prognosis:

• requires life long treatment
• with the right balance of daily medication patients can have a normal lifespan and lead full and productive lives
• over-treatment with glucocorticoids may result in obesity, diabetes, and osteoporosis (3)
• untreated Addison’s disease is fatal and may lead to death quite rapidly due to adrenal crisis

Note:

• the Addison’s Clinical Advisory Panel (ACAP) recommends that all steroid dependant patients should be supplied with a six monthly repeat prescription of their essential steroid medication to avoid risk of running out
• GP’s also should check for possible drug interaction during each time when issuing a new prescription.

Reference:

1. Husebye ES, Pearce SH, Krone NP, et al. Adrenal insufficiency. Lancet. 2021 Feb 13;397(10274):613-29.
2. Bancos I, Hahner S, Tomlinson J, Arlt W. Diagnosis and management of adrenal insufficiency. Lancet Diabetes Endocrinol. 2015 Mar;3(3):216-26
3. Chantzichristos D, Eliasson B, Johannsson G. Management of endocrine disease. Disease burden and treatment challenges in patients with both Addison's disease and type 1 diabetes mellitus. Eur J Endocrinol. 2020 Jul;183(1):R1-R11.