About adrenal insufficiency
The signs and symptoms of adrenal insufficiency are non-specific, develop gradually and can often be mistaken for symptoms of other diseases.1-3
Undetected, however, adrenal insufficiency could lead to an adrenal crisis which in some patients could prove fatal.4
HISTORY
Adrenal insufficiency also known as Addison’s disease was first described by Thomas Addison in 1855 and is characterised by inadequate hormonal production by the adrenal gland.1
Adrenal insufficiency can present either as primary adrenal insufficiency (PAI), due to destruction of the adrenal cortex, or secondary adrenal insufficiency (SAI) or tertiary adrenal insufficiency (TAI), due to lack of adrenocorticotropic hormone or its stimulation by corticotropin-releasing hormone.1
Addison’s disease was an invariably fatal condition in Thomas Addison’s days with most patients dying within a few years of diagnosis. However, discovery of cortisone in the 1940s not only improved the life expectancy of these patients but also had a dramatic effect on their overall quality of life.1
Unfortunately, the diagnosis of adrenal insufficiency remains challenging as there may be an insidious onset of predominantly non-specific symptoms over months or even years.3
Early diagnosis of adrenal insufficiency is important3, because undiagnosed it could lead to an adrenal crisis, which untreated could prove fatal.4
CAUSES
Primary adrenal insufficiency may be inherited or acquired.1 Its most common cause is an autoimmune response, which occurs when the immune system attacks healthy tissues for an unknown reason.5
With primary adrenal insufficiency, the immune system attacks the outer portion of the adrenal glands (the adrenal cortex), where cortisol and aldosterone are produced and therefore does not make enough cortisol.5
Common causes of primary adrenal insufficiency summarised1
Autoimmune:
Isolated, autoimmune polyendocrinopathy syndrome types
1 and 2
Infection:
Tuberculosis, HIV, fungal, viral
Others:
Bilateral adrenal haemorrhage, adrenal metastasis, adrenal infiltration, bilateral adrenalectomy, drug induced
Genetic:
Adrenoleukodystrophy, congenital adrenal hyperplasia, adrenal hypoplasia congenita, adrenocorticotropic hormone, insensitivity syndromes
WHO IS MORE LIKELY TO BE AFFECTED BY PRIMARY ADRENAL INSUFFICIENCY?
Primary adrenal insufficiency is a rare disease with reported numbers rising from 40 to 70 cases per million in Europe in the 1960s to about 100 to 140 cases per million at the beginning of this century.1
Interestingly, this trend seems to be continuing, which could well be due to improved diagnosis over time and increased awareness among physicians.1
Primary adrenal insufficiency:
- Can present at any age, though most patients present between 30 and 50 years1
- Affects women more frequently than men1
- Currently affects about 1 in 14,000 people in the UK, with approximately 9,000 prevalent diagnosed cases and 320 new diagnoses per year6
If you think your patient may have adrenal insufficiency, carry out an early morning cortisol test
References:
- Kumar R, Wassif WS. J Clin Pathol 2022;75:435–442.
- NICE. https://cks.nice.org.uk/topics/addisons-disease/diagnosis/suspecting- addisons-disease/ [Last accessed June 2023].
- Bornstein SR, et al. J Clin Endocrinol Metab 2016;101(2): 364–389.
- NHS. Overview. Addison’s disease. https://www.nhs.uk/conditions/addisons-disease/ [Last accessed June 2023].
- Cleveland Clinic. https://my.clevelandclinic.org/health/diseases/15095-addisons-disease [Last accessed June 2023].
- NICE. https://cks.nice.org.uk/topics/addisons-disease/background-information/prevalence/ [Last accessed June 2023].