Addison's Disease
Endocrine System Confirmed Autoimmune

Addison's Disease

Also known as: Primary Adrenal Insufficiency, Hypoadrenalism, Chronic Adrenocortical Insufficiency

Primary organ/tissue: Adrenal glands (located on top of each kidney)

Overview

Addison's disease is a rare autoimmune disorder in which the adrenal glands do not produce enough of the hormones cortisol and aldosterone. These hormones are essential for regulating metabolism, blood pressure, and stress responses. The disease develops gradually and can become life-threatening if untreated, especially during times of physical stress or illness.

Symptoms

Chronic fatigue; Muscle weakness; Unexplained weight loss; Loss of appetite; Low blood pressure (can cause fainting); Salt cravings; Nausea, vomiting, or abdominal pain; Hyperpigmentation (darkening) of skin, especially in scars or skin creases; Depression or irritability; Low blood sugar (especially in children); Irregular or absent menstrual periods in women.

Causes

Most often caused by autoimmune destruction of the adrenal cortex (70-90% of cases in developed countries). Other causes include infections (e.g., tuberculosis, HIV), genetic disorders (e.g., congenital adrenal hyperplasia), cancer metastasis to adrenal glands, or bleeding into the adrenal glands.

Diagnosis

ACTH stimulation test (gold standard); Blood tests for low cortisol and high ACTH levels; Electrolyte panel showing low sodium and high potassium; Antibodies to 21-hydroxylase (specific marker in autoimmune Addison's); Imaging (CT scan) of adrenal glands.

Treatment

Hormone replacement therapy is lifelong: Hydrocortisone, prednisone, or dexamethasone to replace cortisol; Fludrocortisone to replace aldosterone; Emergency treatment with injectable corticosteroids for adrenal crisis; Stress dosing of steroids during illness, surgery, or injury; Medical ID bracelet recommended.

Prognosis

With proper treatment and education, most people live normal lives. Without treatment, adrenal crisis can be fatal. Patients must be vigilant about stress dosing, staying hydrated, and recognizing symptoms of low cortisol.

Prevalence

Estimated 100-140 cases per million in the general population. Affects both men and women equally. Most common between ages 30-50, but can occur at any age.

Autoantibodies

AutoantibodyNotes
21-hydroxylase antibodies Present in autoimmune Addison''s
Other antibodies May also be seen if part of autoimmune polyglandular syndrome (APS)

Citations

  1. Ten, S., New, M., & Maclaren, N. (2001). Addison''s disease 2001. The Journal of Clinical Endocrinology & Metabolism, 86(7), 2909-2922.
  2. NIH - National Institute of Diabetes and Digestive and Kidney Diseases.
  3. Betterle, C., & Morlin, L. (2011). Autoimmune Addison''s disease. Endocrine Development, 20, 161-172.
  4. Mayo Clinic. Addison''s disease overview.