Addison's disease: primary adrenal insufficiency and management

Typical features and clinical presentation

Cortisol is a stress hormone that affects metabolism, blood pressure and the body’s response to illness. Basic descriptions of hormones and cortisol are discussed in sources such as hormone and cortisol. When cortisol levels are low, people commonly experience chronic fatigue, muscle weakness, poor appetite, and weight loss. Low steroid levels also impair maintenance of blood pressure, producing dizziness and fainting on standing. In primary disease, reduced aldosterone can lead to salt loss, low blood volume and abnormal electrolytes.

Symptoms, signs and clues

• Progressive fatigue, weakness and weight loss.
• Low blood pressure, especially on standing; lightheadedness.
• Darkening of the skin (hyperpigmentation) in scars, creases and mucous membranes in primary disease.
• Salt cravings and gastrointestinal symptoms such as nausea or abdominal pain.
• Disorders of metabolism: impaired handling of insulin and disruptions in processing of proteins and carbohydrates.

Causes and diagnostic approach

In many regions the most common cause of primary Addison's disease is autoimmune destruction of the adrenal cortex; historically, infections such as tuberculosis were a major cause. Secondary adrenal insufficiency can result from deficient production of ACTH by the pituitary, often after long-term steroid use or pituitary disease. Diagnosis typically combines clinical features with laboratory tests: low cortisol, elevated ACTH in primary disease, and dynamic testing such as the ACTH stimulation test. Basic laboratory abnormalities may include low sodium and sometimes high potassium. For more on immune and inflammatory mechanisms see immune system and inflammation.

Treatment and long-term management

Replacement of deficient hormones is the foundation of treatment. Glucocorticoid replacement (commonly hydrocortisone or equivalent) restores cortisol action, while many patients with primary disease also need a mineralocorticoid (fludrocortisone) to maintain sodium and blood pressure. Patients are taught “sick day” rules to increase steroid doses during illness, injury or surgery and to carry emergency glucocorticoid injections. Medical identification and regular follow-up with an endocrinologist reduce the risk of an Addisonian (adrenal) crisis, an acute, severe drop in blood pressure and consciousness that requires prompt parenteral steroids and supportive care.

Prognosis, prevention and notable facts

With appropriate replacement therapy and education, most people with Addison's disease lead active lives, although lifelong treatment and awareness are required. Distinguishing primary from secondary adrenal insufficiency is important because mineralocorticoid replacement is usually required only in primary disease. Historical notes and public figures associated with the condition are sometimes mentioned in summaries; for broader context see blood pressure resources and general overviews at hormone or clinical reference pages such as the endocrine system and condition summaries at cortisol. For patient-focused materials, clinical care pathways and emergency instructions consult trusted health organizations and specialist services at inflammation, immune system and endocrine clinics (proteins and carbohydrates metabolism resources can also be relevant).

Further reading and resources: introductory endocrine texts and reputable clinical guidelines use accessible summaries; see professional and patient information indexed by specialty services or regional health systems at entries such as Thomas Addison, John F. Kennedy and general pages on insulin and metabolic care. Emergency preparedness and correct medication use remain the most important practical measures for people living with Addison's disease.