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Internal Medicine›Condition·Updated Apr 17, 2026·v1

Addison Disease

Addison disease is the primary failure of the adrenal cortex, most commonly due to autoimmunity. It presents with fatigue, hyperpigmentation, and electrolyte imbalances. Diagnosis is confirmed via ACTH stimulation testing. Treatment requires lifelong replacement of glucocorticoids and mineralocorticoids, with mandatory stress-dosing during illness.

High Evidence191 references·831 words·4 min read·v1
EndocrinologyAdrenal InsufficiencyAddison DiseaseAdrenal Crisis

Quick Reference

RxDrug of choiceHydrocortisone (15-25 mg/day divided)
AltAlternativesPrednisone (3-5 mg/day), Fludrocortisone (for mineralocorticoid replacement)
⊘AvoidAbrupt cessation of steroids, Etomidate (can worsen adrenal suppression)
DxTest of choiceACTH Stimulation Test (250 mcg Cosyntropin)
ScKey scoreAddiQoL (Quality of Life in Addison's Disease questionnaire)
→When to referAll suspected cases for diagnostic confirmation and lifelong management plan
Addison disease is a permanent loss of adrenal function requiring lifelong steroid replacement and strict adherence to stress-dosing protocols to avoid fatal adrenal crisis.
Addison disease, or primary adrenal insufficiency (PAI), is a life-threatening endocrine disorder characterized by the failure of the adrenal cortex to produce sufficient glucocorticoids (cortisol), mineralocorticoids (aldosterone), and androgens. In developed nations, the most common cause is autoimmune destruction (autoimmune adrenalitis), while tuberculosis remains a leading cause globally. Because the adrenal glands have a massive functional reserve, clinical symptoms often remain latent until over 90% of the cortex is destroyed, frequently presenting as an insidious progression of fatigue, weight loss, and hyperpigmentation.

Overview and Recommendations

Background

  • •Recognize Addison disease as the primary form of adrenal insufficiency (PAI), where the pathology resides within the adrenal glands themselves, distinguishing it from secondary (pituitary) or tertiary (hypothalamic) failure. This distinction is critical because PAI involves a global loss of all three adrenal steroid layers, whereas secondary forms typically preserve mineralocorticoid function.
  • •Identify autoimmune adrenalitis (AAD) as the etiology in 80-90% of cases in Western countries, often occurring as part of (APS) Type 1 or Type 2. In these patients, the immune system targets the 21-hydroxylase enzyme, leading to progressive lymphocytic infiltration and cortical atrophy.
  • •Consider non-autoimmune etiologies in specific contexts, such as infectious adrenalitis (tuberculosis, CMV in HIV/AIDS), bilateral adrenal hemorrhage (often associated with ), or genetic disorders like X-linked (X-ALD) in young males.
  • •Understand the physiological impact of hormonal loss, where cortisol deficiency leads to impaired gluconeogenesis and hypotension, while aldosterone deficiency causes renal salt wasting, , and . The loss of adrenal androgens is most clinically significant in women, manifesting as a loss of axillary/pubic hair and reduced libido.
  • •Anticipate the risk of an adrenal crisis (AC), which occurs at a rate of 5-10 events per 100 patient-years. This is an endocrine emergency triggered by infection, surgery, or trauma, requiring immediate recognition to prevent circulatory collapse and death.

Evaluation

  • •Suspect Addison disease in any patient presenting with the classic triad of profound fatigue, unexplained weight loss, and hyperpigmentation. Hyperpigmentation is most visible in skin creases, recent scars, and the buccal mucosa, resulting from high ACTH levels cross-reacting with melanocortin-1 receptors.
  • •Ask specifically about salt craving, which is a hallmark of mineralocorticoid deficiency, and gastrointestinal symptoms such as nausea, vomiting, or vague abdominal pain that may mimic an acute abdomen or eating disorder.
  • •Examine for orthostatic hypotension, defined as a systolic drop >20 mmHg or diastolic drop >10 mmHg upon standing, reflecting the volume depletion characteristic of aldosterone deficiency.
  • •Order a morning (8 AM) serum cortisol and plasma ACTH as the initial screening step. A cortisol level < 3 mcg/dL (80 nmol/L) is highly suggestive of insufficiency, while a level > 15 mcg/dL (414 nmol/L) generally rules it out.
  • •Confirm the diagnosis with a high-dose (250 mcg) ACTH stimulation test (Cosyntropin test). Administer 250 mcg of ACTH IV or IM and measure cortisol at 30 and 60 minutes; a peak cortisol response < 18 mcg/dL (500 nmol/L) confirms adrenal insufficiency.
  • •Differentiate primary from secondary insufficiency by checking the baseline ACTH level. In Addison disease, ACTH will be significantly elevated (often > 100 pg/mL), whereas it will be low or inappropriately normal in pituitary-related failure.
  • •Evaluate for mineralocorticoid deficiency by measuring plasma renin activity and aldosterone. In PAI, you will typically find elevated renin with low or undetectable aldosterone levels.
  • •Screen for common electrolyte derangements, specifically (found in 90% of patients) and (found in 65% of patients), along with hypoglycemia and mild metabolic acidosis.
  • •Determine the etiology by testing for 21-hydroxylase antibodies (anti-21-OH). If positive, the diagnosis is autoimmune adrenalitis; if negative, further workup is required.
  • •Perform a CT scan of the adrenal glands if antibodies are negative or if an infectious/hemorrhagic cause is suspected. Look for adrenal calcifications (suggestive of tuberculosis) or enlarged, hemorrhagic glands.
  • •Screen male patients with negative antibodies for X-linked adrenoleukodystrophy by measuring very-long-chain fatty acids (VLCFAs), as PAI may be the only presenting sign before neurological symptoms emerge.

Management

  • •Administer glucocorticoid replacement as the cornerstone of therapy, typically using Hydrocortisone 15–25 mg daily, divided into two or three doses (e.g., 10 mg on waking, 5 mg at lunch, and 2.5 mg in the late afternoon) to mimic the natural circadian rhythm.
  • •Use longer-acting glucocorticoids like Prednisone 3–5 mg once daily as an alternative for patients with poor compliance or those who experience "wear-off" symptoms between hydrocortisone doses, though monitoring for over-replacement is more difficult.
  • •Initiate mineralocorticoid replacement with Fludrocortisone 0.05–0.2 mg daily to maintain normovolemia and normal potassium levels. Unlike glucocorticoids, the dose does not usually need to be increased during minor illness.
  • •Educate the patient on "sick day rules," which involve doubling or tripling the oral glucocorticoid dose during febrile illnesses or minor procedures to prevent progression to an adrenal crisis.
  • •Provide an emergency injection kit containing 100 mg of Hydrocortisone (e.g., Solu-Cortef) and train the patient and their family on IM injection techniques for use during severe vomiting, trauma, or altered consciousness.
  • •Manage an acute adrenal crisis immediately with 100 mg IV Hydrocortisone bolus, followed by 200 mg per 24 hours (either as a continuous infusion or 50 mg every 6 hours), alongside aggressive isotonic saline resuscitation.
  • •Avoid using Dexamethasone for chronic maintenance if possible, as its high potency and lack of mineralocorticoid activity increase the risk of Cushingoid side effects and electrolyte imbalances.
  • •Consider DHEA replacement (25–50 mg daily) in women who report persistently low libido, impaired mood, or low energy despite optimized glucocorticoid and mineralocorticoid therapy.
  • •Adjust doses during pregnancy by increasing the hydrocortisone dose by 20–50% during the third trimester to account for increased cortisol-binding globulin and physiological demand.
  • •Implement perioperative stress dosing: for major surgery, administer 100 mg IV Hydrocortisone at induction, followed by 200 mg/24 hours for 48–72 hours, tapering rapidly back to maintenance as the patient recovers.
  • •Monitor for signs of over-replacement, such as weight gain, insomnia, peripheral edema, or hypertension, and adjust doses to the lowest effective amount to preserve bone mineral density.
  • •Refer all patients to an endocrinologist for long-term management and ensure they wear a medical alert bracelet or carry a steroid emergency card at all times.
  • •Discharge patients only after they demonstrate the ability to self-administer emergency injections and can articulate the specific triggers for seeking emergency care.

Board Review — High Yield

  • •Hyperpigmentation — Caused by high ACTH cross-reacting with MC1R receptors on melanocytes.
  • •21-hydroxylase antibodies — The most specific marker for autoimmune adrenalitis in adults.
  • •Hyponatremia and Hyperkalemia — The classic electrolyte pattern of primary (but not secondary) adrenal insufficiency.
  • •Salt Craving — A specific symptom of mineralocorticoid deficiency found in PAI.
  • •Adrenal Crisis — Presents as refractory shock; treat with IV fluids and 100mg IV Hydrocortisone immediately.
  • •X-linked Adrenoleukodystrophy — Consider in young males with PAI and negative antibodies; check VLCFAs.
  • •Waterhouse-Friderichsen Syndrome — Acute adrenal failure due to bilateral hemorrhage, often from Neisseria meningitidis.

Deep Dive — Evidence Details

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