Addison's disease

Addison's disease or primary adrenocortical deficiency results from destruction of the adrenal cortex. Glucocorticoid, mineralocorticoid and sex steroid production are all reduced.

Primary adrenal insufficiency is a rare condition with an estimated incidence of 4:1,000,000 per year in developed countries and an overall prevalence ranging from 100 to 200 cases per million in Western countries.(1)

• may occur at any age but commonly seen between the ages of 30-50 years (2)
• women are affected more than men (3)

Autoimmune adrenalitis accounts increasingly for the condition as tuberculosis has become less common.

The disease may remain subclinical for a long period of time (4).

Notes (5):

• primary and secondary adrenal insufficiency share many clinical features - however, they differ in that only primary adrenal insufficiency is characterized by mineralocorticoid deficiency and by hyperpigmentation

Reference:

1. Barthel A, Benker G, Berens K, et al. An update on Addison's disease. Exp Clin Endocrinol Diabetes. 2019 Feb;127(2-03):165-75.
2. Wass, J., Howlett, T., Arlt, W. et al. Diagnosing Addison's: a guide for GPs. Addison's Disease Self Help Group 2009
3. Gunna S, Singh M, Pandey R, et al. Etiology, clinical characteristics and mortality among Indian patients with Addison's disease. Endocr Connect. 2023 Feb 11;12(3):e220439.
4. Betterle C, Presotto F, Furmaniak J. Epidemiology, pathogenesis, and diagnosis of Addison's disease in adults. J Endocrinol Invest. 2019 Dec;42(12):1407-33.
5. Husebye ES, Pearce SH, Krone NP, et al. Adrenal insufficiency. Lancet. 2021 Feb 13;397(10274):613-29.