From the Guidelines
Addison's disease is primarily caused by autoimmune adrenalitis, accounting for approximately 85% of cases in Western Europe, where the body's immune system mistakenly attacks and destroys the adrenal cortex. The diagnosis of an autoimmune cause is based on measurements of circulating 21OH-Ab 1. Other causes of primary adrenal insufficiency (PAI) include tuberculosis, adrenal hemorrhage, and genetic disorders 1. In patients without 21OH-Ab, a more thorough investigation must be undertaken to determine the underlying cause of the disease 1.
Causes of Addison's Disease
- Autoimmune adrenalitis: the most common cause, accounting for approximately 85% of cases in Western Europe 1
- Tuberculosis: a historical leading cause, now less common in developed countries
- Fungal infections
- Cancer that has spread to the adrenal glands
- Bleeding into the adrenal glands
- Certain medications like ketoconazole and etomidate that can inhibit cortisol production
- Genetic disorders such as adrenoleukodystrophy and congenital adrenal hyperplasia
Diagnosis and Treatment
The diagnosis of Addison's disease involves confirming primary adrenal insufficiency and determining the underlying cause 1. Treatment typically involves hormone replacement therapy with glucocorticoids and mineralocorticoids to replace the hormones the adrenal glands can no longer produce. The goal of treatment is to improve the quality of life and reduce the risk of premature death from adrenal crises 1.
From the Research
Causes of Addison's Disease
Addison's disease is typically caused by the autoimmune destruction of the adrenal cortex, leading to deficiencies in glucocorticoids, mineralocorticoids, and adrenal androgens 2, 3. The most common cause of Addison's disease is autoimmune adrenalitis, which results from the destruction of the adrenal cortex 3.
Other Causes
Other causes of Addison's disease include:
- Infectious diseases
- Neoplastic disorders
- Genetic disorders
- Iatrogenic conditions, such as adrenal hemorrhage associated with anticoagulants, drugs affecting glucocorticoid synthesis, action or metabolism, and some novel anti-cancer checkpoint inhibitors 4
- Autoimmune diseases, with up to 50% of patients developing another autoimmune disorder during their lifetime 3
Key Factors
Key factors that contribute to the development of Addison's disease include:
- Autoimmune disorders
- Genetic predisposition
- Infections
- Trauma to the adrenal glands
- Certain medications, such as anticoagulants and anti-cancer drugs 4, 5
Diagnosis and Treatment
Diagnosis of Addison's disease is often delayed due to non-specific symptoms, and treatment requires replacement of mineralocorticoids and glucocorticoids 2, 3. Patients with Addison's disease require lifelong replacement therapy and must be educated on how to manage their condition to prevent adrenal crisis 4, 5.