Addison’s disease is primary adrenal insufficiency – the destruction or dysfunction of the adrenal cortex itself, producing a deficiency of both cortisol and aldosterone. It is rare: in developed countries it affects about 100 to 140 people per million, and autoimmune disease causes 8 or 9 of every 10 cases (NIDDK). Secondary adrenal insufficiency is more common, at 150 to 280 per million. The hallmark finding that distinguishes primary from secondary adrenal insufficiency is hyperpigmentation: excess ACTH (the pituitary’s response to low cortisol) exerts MSH-like activity on melanocytes, producing bronze or darkening of the skin and mucous membranes. Secondary adrenal insufficiency involves pituitary ACTH deficiency and does not produce hyperpigmentation. The life-threatening complication, Addisonian crisis, requires immediate parenteral hydrocortisone and fluid resuscitation. Crises are not rare even in well-educated patients: a prospective study of 423 patients found 8.3 crises per 100 patient-years and 0.5 crisis-related deaths per 100 patient-years (Hahner 2015). This reference covers pathophysiology, clinical presentation, laboratory findings, adrenal crisis management, nursing interventions, medications, patient education, and six NCLEX-style questions.
Use this page alongside the Cushing’s syndrome nursing reference for the full cortisol excess vs. deficiency comparison, the electrolyte imbalances reference for hyponatremia and hyperkalemia management, the diabetes mellitus nursing reference for hypoglycemia management, and the nursing lab values cheat sheet for cortisol and ACTH interpretation.
| Fast-scan summary | Key fact |
|---|---|
| Definition | Primary adrenal insufficiency – adrenal cortex destruction → cortisol + aldosterone deficiency |
| Most common cause | Autoimmune adrenalitis (80–90% of cases in developed countries, per NIDDK) |
| Distinguishing sign | Hyperpigmentation – bronze darkening of skin, buccal mucosa, scars, palmar creases, pressure points (primary only; excess ACTH → MSH-like stimulation) |
| Electrolyte pattern | Hyponatremia + hyperkalemia (aldosterone deficiency → Na+ wasting, K+ retention); hyponatremia also occurs in secondary disease, hyperkalemia does not |
| Gold standard diagnostic test | ACTH (cosyntropin) stimulation test – 250 mcg IV, cortisol at baseline and at 30 or 60 min; a peak below 18 mcg/dL (500 nmol/L, assay dependent) indicates insufficiency (Endocrine Society 2016) |
| Lab pattern in primary | Low cortisol + HIGH ACTH (more than twice the upper reference limit); in secondary: low cortisol + low or inappropriately normal ACTH |
| Chronic cortisol replacement | Hydrocortisone 15–25 mg/day in 2–3 divided doses (largest dose in morning) |
| Mineralocorticoid replacement | Fludrocortisone, starting dose 50–100 mcg/day in adults, with no salt restriction (primary Addison's only – secondary does not require mineralocorticoid) |
| Adrenal crisis treatment | Hydrocortisone 100 mg IV or IM STAT, then 200 mg/24 h (continuous infusion or 50 mg every 6 h) + 1 L isotonic saline in the first hour |
| Non-negotiable patient education | Sick-day rules + medical alert bracelet + steroid emergency card + emergency hydrocortisone injection kit (Endocrine Society 2016 recs 4.4–4.6) |
Pathophysiology and etiology
The adrenal cortex has three zones, each producing distinct hormones. In Addison’s disease, destruction of the cortex impairs production of glucocorticoids (primarily cortisol, from the zona fasciculata), mineralocorticoids (primarily aldosterone, from the zona glomerulosa), and androgens (primarily DHEA, from the zona reticularis). The medulla, which produces catecholamines (epinephrine, norepinephrine), may also be affected depending on the underlying cause, but the cortisol and aldosterone deficiencies dominate the clinical picture.
When the adrenal cortex is destroyed, cortisol falls. The anterior pituitary detects this via loss of negative feedback and releases large amounts of ACTH in an attempt to drive adrenal output. ACTH shares structural homology with melanocyte-stimulating hormone (MSH): both derive from the precursor proopiomelanocortin (POMC). Excess ACTH binds to melanocortin-1 receptors (MC1R) on melanocytes, stimulating melanin production. This produces the characteristic hyperpigmentation that distinguishes primary from secondary adrenal insufficiency. In secondary adrenal insufficiency, the problem originates at the pituitary (ACTH deficiency), so ACTH is LOW – hyperpigmentation does not occur.
| Cause | Mechanism | Notes |
|---|---|---|
| Autoimmune adrenalitis | Antibodies against 21-hydroxylase enzyme destroy the adrenal cortex | Most common cause (80–90% in developed countries, per NIDDK); positive anti-21-hydroxylase antibodies confirm an autoimmune cause; often part of autoimmune polyendocrine syndrome (APS) – co-occurs with thyroid disease, type 1 diabetes, celiac disease |
| Tuberculosis | Granulomatous destruction of the adrenal glands; may calcify on imaging | Leading infectious cause worldwide; consider in patients from endemic regions or with prior TB; bilateral adrenal calcification on CT is a classic finding |
| Fungal infections | Histoplasma, Cryptococcus, Blastomyces invade adrenal tissue | Associated with immunocompromised states (HIV/AIDS); histoplasmosis is a classic cause in endemic regions (Ohio/Mississippi River valleys) |
| Metastatic cancer | Bilateral adrenal metastases destroy functional cortical tissue (typically requires >90% destruction for insufficiency) | Most common primary tumors: lung, breast, colon, kidney, melanoma; often an incidental finding on staging CT |
| Adrenal hemorrhage | Bilateral adrenal infarction, often from anticoagulant therapy, septicemia (Waterhouse-Friderichsen syndrome with meningococcemia), or antiphospholipid syndrome | Waterhouse-Friderichsen syndrome: acute bilateral adrenal hemorrhage in septicemia – dramatic onset, often fatal without early recognition |
| Medications | Etomidate, ketoconazole, osilodrostat, metyrapone (inhibit steroidogenesis); mitotane (adrenolytic); rifampin, phenytoin, carbamazepine (CYP3A4 induction speeds cortisol clearance) | Enzyme inhibitors and mitotane can cause adrenal insufficiency on their own. Inducers leave the adrenal intact; they can unmask insufficiency or push a treated patient into crisis because the same hydrocortisone dose no longer lasts |
| Congenital adrenal hyperplasia (CAH) | Enzyme deficiency (most often 21-hydroxylase) impairs cortisol and, in the salt-wasting form, aldosterone synthesis from birth | A primary adrenal insufficiency distinct from autoimmune Addison's; the salt-wasting form usually presents in the first weeks of life (US newborn screening tests for it) |
Primary vs secondary vs tertiary adrenal insufficiency
| Feature | Primary (Addison's) | Secondary (pituitary) | Tertiary (hypothalamic) |
|---|---|---|---|
| Problem location | Adrenal cortex | Anterior pituitary | Hypothalamus |
| Primary hormone deficit | Cortisol AND aldosterone (adrenal cannot produce them); ACTH and CRH are elevated in response | ACTH (pituitary cannot produce it); CRH elevated in response; aldosterone preserved | CRH (hypothalamus cannot produce it); ACTH low as a consequence; aldosterone preserved |
| Cortisol | Low | Low | Low |
| ACTH level | HIGH (pituitary drives compensation) | Low | Low |
| Aldosterone | Low (adrenal destruction) | Normal (RAAS intact – renin drives aldosterone independently) | Normal |
| Hyperpigmentation | YES – excess ACTH stimulates MC1R | No – ACTH is low | No – ACTH is low |
| Salt craving / hypovolemia | Yes – aldosterone deficiency | Mild – SIADH-like hyponatremia possible but no aldosterone deficit | Mild |
| Mineralocorticoid replacement needed | Yes (fludrocortisone) | No | No |
| Most common cause | Autoimmune adrenalitis | Long-term exogenous steroid use (HPA axis suppression) | Long-term exogenous steroid use (CRH suppression) |
Clinical presentation
Addison’s disease develops insidiously over months to years. Because most of the adrenal cortex (classically quoted as about 90%) must be destroyed before overt insufficiency appears, patients often present with vague symptoms that are initially attributed to depression, chronic fatigue, or gastrointestinal illness. In more than half of patients, the diagnosis is only made after they present in adrenal crisis (Society for Endocrinology 2016). Recognizing the constellation – fatigue + weight loss + hyperpigmentation + orthostatic hypotension + salt craving – earlier is essential for both clinical practice and NCLEX.
Glucocorticoid deficiency (cortisol) manifestations:
- Fatigue and generalized weakness (most common, often profound)
- Anorexia, nausea, vomiting, abdominal pain
- Weight loss
- Hypoglycemia (cortisol is a counter-regulatory hormone – its absence reduces gluconeogenesis); clinically important mainly in children, and in adults who also take insulin
- Depression, irritability, cognitive difficulties
- Amenorrhea and loss of libido (adrenal androgens depleted, affecting sex hormone balance)
- Increased sensitivity to insulin (hypoglycemia risk particularly in patients also on insulin)
Mineralocorticoid deficiency (aldosterone) manifestations:
- Hyponatremia – aldosterone normally promotes Na+ reabsorption in the distal nephron; without it, Na+ is wasted in the urine
- Hyperkalemia – aldosterone normally drives K+ excretion; without it, K+ accumulates
- Hypovolemia and hypotension – Na+ wasting leads to volume depletion
- Orthostatic hypotension – drop in systolic BP ≥20 mmHg and/or diastolic BP ≥10 mmHg on standing
- Back and leg cramps or spasms – commonly reported in crisis and easy to misattribute (Society for Endocrinology 2016)
- Salt craving – a classic history clue; patients describe craving salty foods
- Dizziness on standing, palpitations
Androgen deficiency manifestations (more prominent in women):
- Loss of axillary and pubic hair (adrenal androgens are the primary source in women)
- Decreased libido
- Dry skin
Hyperpigmentation (primary Addison’s only): This is the most NCLEX-important physical finding. Excess ACTH activates MC1R on melanocytes, producing bronze or tan discoloration, particularly prominent at:
- Sun-exposed areas (face, neck, dorsum of hands)
- Pressure points (elbows, knees, knuckles)
- Skin folds (axillae, groin)
- Buccal mucosa and gingiva (darkening of the inner cheeks is a classic clue, though oral pigmentation also occurs normally in many people with darker skin)
- Scars and areolae
- Palmar creases
New darkening of existing scars is a classic NCLEX clue – scars formed before the disease developed now darken as ACTH rises.
Laboratory findings
| Lab value | Expected finding | Mechanism | Clinical significance |
|---|---|---|---|
| Serum cortisol (AM) | Low; a morning cortisol <5 mcg/dL (140 nmol/L) together with a high ACTH is a preliminary result suggestive of insufficiency when a stimulation test is not yet feasible | Adrenal cortex cannot produce adequate cortisol | Draw early in the morning, near the diurnal peak; a single low value is not diagnostic, so confirm with a cosyntropin test (Endocrine Society 2016 rec 2.3). The 18 mcg/dL cutoff applies to the stimulated peak only |
| Plasma ACTH | High in primary: more than twice the upper limit of the reference range in a patient with confirmed cortisol deficiency (Endocrine Society 2016 rec 2.4) | Loss of cortisol negative feedback drives pituitary ACTH hypersecretion | High ACTH + low cortisol = primary; low or inappropriately normal ACTH + low cortisol = secondary/tertiary. Draw with the baseline cortisol sample |
| ACTH stimulation test (gold standard) | Cortisol fails to reach 18 mcg/dL (500 nmol/L) at 30 or 60 min after cosyntropin 250 mcg IV (125 mcg under age 2; 15 mcg/kg in infants) | Adrenal cortex cannot respond to ACTH stimulation | The cutoff is assay dependent, and newer, more specific cortisol assays use lower thresholds; follow your lab's reference. The low-dose (1 mcg) test is suggested only when cosyntropin is in short supply (Endocrine Society 2016 recs 2.1–2.2) |
| Serum sodium (Na+) | Low (hyponatremia) in most patients, though a normal sodium does not exclude the diagnosis | Aldosterone deficiency → sodium wasting via the kidney; cortisol deficiency also raises ADH, which is why secondary disease can cause hyponatremia too | Can cause confusion, seizures at very low levels; correct gradually (rapid correction risks osmotic demyelination once cortisol is replaced and free-water excretion resumes) |
| Serum potassium (K+) | High (hyperkalemia) in primary disease only; often absent at diagnosis | Aldosterone normally drives K+ secretion in distal nephron; without it, K+ accumulates | Monitor for cardiac dysrhythmias (peaked T waves, widened QRS); extreme hyperkalemia is life-threatening |
| Serum glucose | Low (hypoglycemia), mainly in children | Cortisol promotes gluconeogenesis and glycogenolysis; its absence impairs glucose maintenance | The Society for Endocrinology notes hypoglycemia occurs primarily in affected children, where it can cause lasting neurological harm; in adults, watch most closely in those also taking insulin (a falling insulin requirement is a clue) |
| Serum aldosterone | Low | Aldosterone is produced by the adrenal cortex (zona glomerulosa) – destroyed in primary disease | Measured alongside plasma renin activity (PRA); in primary Addison's: low aldosterone + high PRA (renin is upregulated trying to drive aldosterone) |
| Plasma renin activity (PRA) | High | Low aldosterone → volume depletion → renin/angiotensin system activated | High renin + low aldosterone = mineralocorticoid deficiency; the Endocrine Society recommends measuring both together at diagnosis (rec 2.5) |
| Blood urea nitrogen (BUN) | Elevated (pre-renal azotemia) | Volume depletion from aldosterone deficiency reduces renal perfusion | BUN:creatinine ratio >20:1 suggests pre-renal cause |
| Eosinophil count | Elevated (eosinophilia) | Cortisol normally suppresses eosinophils; cortisol deficiency removes this suppression | Eosinophilia in the context of chronic fatigue and hyponatremia is a subtle clue to adrenal insufficiency |
| Lymphocyte count and hemoglobin | Lymphocytosis; normochromic anemia | Cortisol normally suppresses lymphocytes; its absence allows lymphocytosis | The Society for Endocrinology lists normochromic anemia and, sometimes, lymphocytosis and eosinophilia: soft signs that cannot make the diagnosis on their own |
| Metabolic acidosis | Possible mild hyperchloremic (normal anion gap) metabolic acidosis | Aldosterone deficiency impairs H+ and K+ secretion in the distal nephron → H+ retention | Usually mild: low HCO3 with a normal anion gap; pH may stay in range. See [ABG interpretation guide](/nursing-tips/abg-interpretation/) |
| Anti-21-hydroxylase antibodies | Positive in autoimmune Addison's | Autoantibodies target 21-hydroxylase, the enzyme essential for cortisol and aldosterone synthesis | The first test for etiology once primary insufficiency is confirmed; a positive result supports an autoimmune cause, and the ACTH stimulation test is still needed for the functional diagnosis. A negative result prompts adrenal CT and other workup (Endocrine Society 2016 rec 2.6) |
Adrenal crisis (Addisonian crisis)
Adrenal crisis is the most dangerous and time-critical complication of Addison’s disease. It is a life-threatening emergency characterized by severe hypotension, cardiovascular collapse, and acute cortisol deficiency. Without immediate treatment, it is fatal. Nursing recognition and rapid response are essential.
Triggers
Any significant physiologic stressor can precipitate a crisis in a patient with adrenal insufficiency, because these patients cannot mount the normal cortisol stress response:
- Infection (the leading trigger – in a prospective study, gastrointestinal infection and fever were each behind about 20% of crises, Hahner 2015)
- Surgery or invasive procedures (without stress-dose steroid coverage)
- Trauma
- Missed steroid doses (skipping scheduled hydrocortisone, vomiting unable to keep medication down)
- Sudden steroid discontinuation (including in patients on long-term exogenous steroids who develop HPA axis suppression; the 2024 ESE/ES guideline says to suspect crisis in any recent steroid user with hemodynamic instability, vomiting or diarrhea, whatever the drug, route or dose)
- New thyroid hormone or hyperthyroidism (levothyroxine started before cortisol is replaced, or thyrotoxicosis, speeds cortisol clearance)
- Gastrointestinal illness with vomiting/diarrhea (unable to absorb oral steroids)
- Emotional stress (about 20% of crises in the same study), plus severe pain, heat, strenuous exercise and pregnancy
- Unexplained – about 7% of crises had no identifiable trigger
Signs and symptoms of adrenal crisis
- Severe hypotension – systolic BP often <90 mmHg; may progress to shock
- Profound weakness and lethargy – patient may be unable to stand or move
- Severe nausea, vomiting, abdominal pain – can mimic an acute abdomen
- Fever – may come from the precipitating infection or from the crisis itself
- Altered mental status – confusion, disorientation, coma in severe cases
- Tachycardia – compensatory response to volume depletion
- Hypoglycemia – severe, may cause seizures
- Severe hyponatremia and hyperkalemia – electrolyte crisis can cause cardiac dysrhythmia
Immediate nursing actions for adrenal crisis
- Call for immediate provider/rapid response – this is a life-threatening emergency requiring IV hydrocortisone immediately
- Establish IV access – large-bore IV (16–18 gauge); if IV access is delayed, hydrocortisone can be given IM
- Obtain STAT labs – serum cortisol, ACTH, BMP (Na+, K+, glucose, BUN, creatinine), CBC with differential, blood cultures if infection suspected. Draw labs before giving steroids if possible – but do not delay steroids if labs will take time
- Administer hydrocortisone 100 mg IV (or IM) immediately – this is the cornerstone treatment; give it without waiting for cortisol results if clinical suspicion is high (Endocrine Society 2016 recs 1.3 and 4.1). There are no adverse consequences to giving it to a patient who turns out not to have adrenal insufficiency (Society for Endocrinology 2016)
- Initiate IV fluid resuscitation – 1,000 mL of isotonic saline (0.9% NaCl) over the first hour, then further IV fluid as required (usually 4–6 L in 24 hours). Watch for fluid overload in older adults and in renal impairment (Society for Endocrinology 2016). Add dextrose if the patient is hypoglycemic
- Monitor and treat hypoglycemia – check glucose at presentation and repeatedly; treat per protocol (for example, IV dextrose) if low
- Continuous cardiac monitoring – hyperkalemia can cause dysrhythmias; watch for peaked T waves, widened QRS
- Identify and treat the precipitating cause – blood/urine cultures, broad-spectrum antibiotics if sepsis suspected; check TSH and free T4 (the Society for Endocrinology advises not starting thyroxine for a TSH ≤10 mU/L during crisis, since cortisol deficiency itself can raise TSH)
- Continue hydrocortisone – after the 100 mg bolus: hydrocortisone 200 mg per 24 hours, as a continuous IV infusion or 50 mg IV/IM every 6 hours (children: 50–100 mg/m² per 24 h) (Endocrine Society 2016 rec 4.1). If hydrocortisone is unavailable, prednisolone is the suggested alternative; dexamethasone is the least-preferred option (rec 4.2)
- Position and safety – lay the patient flat (supine) to support cerebral perfusion; passive leg raise may help transiently while fluids are running. Reverse Trendelenburg is head-UP and would worsen hypotension – do not use it here. Fall precautions for orthostasis and altered mental status
Steroid taper post-crisis
Once the patient is stable, oral intake is tolerated, and the precipitating cause is treated, the hydrocortisone dose is tapered back toward maintenance, guided by an endocrinologist. In primary adrenal insufficiency, fludrocortisone (starting dose 100 mcg once daily) is started once the daily hydrocortisone dose falls below 50 mg; above that dose, hydrocortisone’s own mineralocorticoid activity is enough (Society for Endocrinology 2016).
Nursing assessment
Vital signs
- Blood pressure with orthostatic measurements (key assessment): check BP and HR lying, sitting, and standing. A drop of ≥20 mmHg systolic or ≥10 mmHg diastolic on standing, or HR increase ≥20 bpm, confirms orthostatic hypotension. Document all three positions.
- Temperature: fever may indicate infection (a common crisis trigger) or may occur during the crisis itself
- Weight: daily weights detect fluid volume changes; weight loss trend supports chronic adrenal insufficiency
Skin assessment for hyperpigmentation
This is the most NCLEX-critical physical assessment finding in Addison’s disease. Inspect systematically:
- Buccal mucosa and gingiva – new grayish-brown patches on the inner cheeks and gums are a classic sign of primary adrenal insufficiency
- Pressure points – elbows, knees, knuckles, heels
- Skin folds – axillae, groin, abdominal folds
- Sun-exposed areas – face, neck, dorsum of hands and forearms
- Scars – existing scars become hyperpigmented as ACTH rises (a history clue: “my old scars are turning dark”)
- Areolae and genitalia
- Palmar creases
Document baseline color and any changes. In patients with darker baseline skin tones, ask whether the color is new and compare with older photographs; physiologic oral and palmar-crease pigmentation is common, so a change matters more than the finding itself. Secondary adrenal insufficiency tends to produce pale, “alabaster-like” skin instead (Society for Endocrinology 2016).
Electrolyte and glucose monitoring
- Monitor serum Na+, K+, and glucose every 4–8 hours during acute illness or hospitalization
- In chronic outpatient management: the Endocrine Society suggests endocrine review at least annually (infants every 3–4 months), checking for over- and under-replacement, with electrolytes to monitor fludrocortisone, and periodic screening for associated autoimmune disease (thyroid disease, diabetes, premature ovarian failure, celiac disease, B12 deficiency) (recs 3.8, 5.1–5.3)
- Monitor for signs of hyperkalemia: muscle weakness, palpitations, peaked T waves on ECG
- Monitor for hypoglycemia: diaphoresis, tremor, confusion, palpitations, hunger
- Maintain continuous glucose monitoring in patients with concurrent diabetes
Fluid balance
- Strict intake and output during acute illness
- Daily weights (same scale, same time of day)
- Assess for dehydration: skin turgor, mucous membrane moisture, urine output (target ≥0.5 mL/kg/hr), urine specific gravity
Nursing interventions
| Problem | Nursing interventions | Rationale |
|---|---|---|
| Fluid volume deficit (aldosterone deficiency) | Administer IV fluids as ordered (isotonic NS preferred); monitor I&O strictly; daily weights; assess orthostatic BP q shift; administer fludrocortisone as ordered | Aldosterone deficiency causes sodium and water wasting; volume repletion corrects hypovolemia; fludrocortisone replaces mineralocorticoid action |
| Risk for electrolyte imbalance | Monitor serum Na+, K+, and glucose frequently; cardiac monitoring for hyperkalemia dysrhythmias; administer ordered electrolyte supplements (Na+ replacement in hyponatremia); hold potassium supplementation; assess neuro status for signs of hyponatremia-related cerebral edema | Aldosterone deficiency → hyponatremia + hyperkalemia; cortisol deficiency → hypoglycemia; dysrhythmia risk with K+ >6.0 mEq/L |
| Activity intolerance (profound fatigue) | Schedule nursing activities around rest periods; assist with ADLs; implement fall precautions; educate patient on energy conservation techniques; assess fatigue level on validated scale | Cortisol deficiency causes profound weakness and fatigue; orthostatic hypotension elevates fall risk; cortisol replacement takes days to restore energy |
| Risk for hypoglycemia | Monitor blood glucose during acute illness, and routinely in children and insulin users; have IV dextrose or oral glucose available; administer glucocorticoids with food when possible; educate on hypoglycemia signs and treatment (15-15 rule) | Cortisol promotes gluconeogenesis; deficiency can cause fasting hypoglycemia, most often in children; risk increases with missed meals or illness |
| Risk for adrenal crisis | Monitor for crisis triggers (infection, nausea/vomiting, missed doses); educate patient on sick day rules and crisis recognition; ensure emergency injection kit is available and patient/family trained; have crash cart accessible for hospitalized patients | Any physiologic stressor can precipitate crisis; early recognition and steroid dose escalation are life-saving; a patient who cannot take oral steroids due to vomiting needs IV hydrocortisone immediately |
| Knowledge deficit (steroid management) | Teach medication regimen, sick day rules, stress dosing; provide written steroid ID card; ensure medical alert bracelet is ordered; demonstrate emergency injection kit use; involve family in teaching; document comprehension | Patients must self-manage dose escalation during illness; failure to do so results in adrenal crisis; emergency responders need to know the diagnosis if patient is unconscious |
| Impaired skin integrity (hyperpigmentation and dry skin) | Assess skin systematically at each visit using consistent lighting; document and photograph baseline hyperpigmentation; assess buccal mucosa; use moisturizing skin care; protect from pressure injury in bedridden patients | Persistent or worsening pigmentation can suggest under-replacement and is worth reporting; the Endocrine Society advises adjusting glucocorticoid dose on clinical assessment alone, without hormone levels (recs 3.5–3.6) |
| Body image disturbance | Acknowledge cosmetic impact of hyperpigmentation openly; provide realistic expectations about partial improvement with treatment (ACTH decreases with adequate replacement but hyperpigmentation may persist); refer to counseling if significant psychological distress | Hyperpigmentation affects quality of life; improved cortisol replacement lowers ACTH and can reduce melanocyte stimulation over time |
Medications
| Medication | Class | Dose and schedule | Nursing considerations |
|---|---|---|---|
| Hydrocortisone | Glucocorticoid (cortisol replacement) | Oral: 15–25 mg/day in 2–3 divided doses, largest dose on waking, the next in the early afternoon (Endocrine Society 2016 rec 3.2). Typical split: 10–15 mg on waking + 5–10 mg early afternoon. Prednisolone 3–5 mg/day is an alternative (rec 3.3). For crisis: 100 mg IV or IM, then 200 mg/24 h as a continuous IV infusion or 50 mg IV/IM every 6 hours (rec 4.1) | Largest dose on waking to mimic the diurnal cortisol rhythm (cortisol normally peaks in the early morning). Give with food to prevent GI irritation. Monitor for over-replacement (hypertension, weight gain, hyperglycemia, insomnia, which mirror Cushing's) and under-replacement (fatigue, nausea, postural dizziness). The dose is adjusted on clinical assessment alone (recs 3.5–3.6) |
| Fludrocortisone (Florinef) | Synthetic mineralocorticoid (aldosterone replacement) | Usual dose 0.1 mg (100 mcg) orally once daily; the label range is 0.1 mg three times a week to 0.2 mg daily. The Endocrine Society starting dose is 50–100 mcg in adults, with no salt restriction (rec 3.7) | For PRIMARY Addison's only – secondary adrenal insufficiency retains RAAS function, no mineralocorticoid replacement needed. Monitor clinically and with electrolytes (rec 3.8): over-replacement → hypertension, edema, hypokalemia; under-replacement → salt craving, postural hypotension, hyperkalemia. If hypertension develops, the dose is reduced (rec 3.9; the label suggests 0.05 mg daily), and if BP stays high, antihypertensives are added while fludrocortisone continues (rec 3.10). Extra salt may be needed in hot weather or with heavy sweating |
| Stress-dose hydrocortisone (sick day rules) | Glucocorticoid – dose escalation | Illness with fever, illness needing bed rest or antibiotics: double the daily dose (as a guide, hydrocortisone 40 mg/day in three doses, e.g. 20 mg on rising, 10 mg at midday, 10 mg at 5 PM) for 2–5 days until well. Persistent vomiting or diarrhea: hydrocortisone 100 mg IM IMMEDIATELY (repeat after 6 hours if needed), then emergency care. Procedures under local anesthesia, including dental work: the same 40 mg/day regimen, starting with 20 mg one hour before. Surgery under general or regional anesthesia: 100 mg IV at induction, then 200 mg/24 h by infusion (or 50 mg IV every 6 hours); once eating, a doubled oral dose for 48 hours, then the usual dose (2024 ESE/ES guideline, Table 8) | Teach the "sick day rule" clearly: patients must know the threshold for dose escalation, when to inject themselves, and when to call 911. A common route to a preventable crisis is vomiting that stops oral absorption while the patient delays the injection. The 2024 guideline notes that stress-dose regimens come from clinical practice with little trial evidence, and centers vary |
| Hydrocortisone emergency injection kit | Glucocorticoid – emergency | Hydrocortisone sodium succinate (Solu-Cortef) 100 mg IM, given by the patient or a trained caregiver when vomiting or collapse prevents oral dosing | Every patient should have a kit and be taught to use it (rec 4.6). Solu-Cortef 100 mg comes as an Act-O-Vial, a two-compartment vial: pressing the plunger-stopper mixes the diluent into the powder before the dose is drawn up; it is labeled for IM or IV use. The outer thigh is the usual self-injection site, and the label warns that deltoid injection carries a high rate of subcutaneous atrophy. Verify the kit is not expired at every follow-up. Patient and at least one household member should demonstrate the technique before discharge |
| Prednisolone or dexamethasone (alternatives only) | Glucocorticoid – second-line | Only if hydrocortisone is unavailable: prednisolone is the suggested alternative; dexamethasone is the least-preferred option, used only if no other glucocorticoid is available (Endocrine Society 2016 rec 4.2) | Hydrocortisone is the drug of choice in adrenal crisis and should never be withheld while waiting for a diagnostic test (rec 1.3). Dexamethasone does not cross-react with cortisol immunoassays; that property never justifies delaying hydrocortisone in a suspected crisis. The guideline also advises against dexamethasone for ongoing replacement because dose titration is difficult and Cushingoid side effects are common (rec 3.4). Switch to hydrocortisone as soon as it can be obtained |
Patient education
Patient education in Addison’s disease is life-saving – not merely health-promoting. Patients who understand their disease and know how to manage acute stress can prevent crisis. Patients who do not understand their disease die of preventable adrenal crisis.
Medical alert bracelet and steroid identification card (non-negotiable)
Every patient with Addison’s disease must wear a medical alert bracelet stating “Adrenal Insufficiency: Needs Hydrocortisone in Emergency.” This is not optional. If the patient is unconscious in an emergency department, this bracelet is the only information emergency clinicians have. The bracelet must:
- State the diagnosis (adrenal insufficiency or Addison’s disease)
- State the emergency medication (hydrocortisone)
- Be worn at all times
A steroid identification card (wallet card) complements the bracelet with more detail: diagnosis, maintenance dose, emergency dose (100 mg hydrocortisone IV/IM), prescribing provider contact information.
Sick day rules
Key principle: when the body is stressed, it needs more cortisol. Healthy adrenal glands raise cortisol output several-fold during major illness or surgery. Patients with Addison’s disease cannot do this, so they must increase their dose themselves (Endocrine Society 2016 recs 4.3–4.4). The doses below follow the 2024 ESE/ES guideline (Table 8), which states that stress doses are the same whatever the cause of adrenal insufficiency.
- Fever or illness needing bed rest or antibiotics: Double the daily hydrocortisone dose (as a guide, 40 mg a day in three doses) until well, usually 2–5 days, then return to the usual dose
- Vomiting or inability to take oral medications: This is an emergency. Administer emergency injection (100 mg hydrocortisone IM) immediately, then call 911 or go to the emergency department. Do not wait to see if vomiting resolves.
- Dental procedures, minor procedures under local anesthesia: Take 20 mg hydrocortisone one hour before, then 10 mg six hours after and 10 mg six hours later (40 mg total), continuing if still unwell
- Surgery under general or regional anesthesia, labor, serious trauma: The team gives hydrocortisone 100 mg IV, then 200 mg over 24 hours; make sure the surgical and anesthesia teams know about the diagnosis in advance
When to call 911 vs. when to call the provider
Call 911 (emergency) if:
- Vomiting and cannot take oral medications (inject first, then call)
- Severe weakness, unable to stand
- Confusion or altered mental status
- Systolic BP <90 mmHg
- Loss of consciousness
Call the provider (within hours) if:
- Fever >100.4°F with no obvious cause
- Increasing fatigue or weakness despite dose adjustment
- Signs of over-replacement: swelling, weight gain, blood pressure elevation
Never abruptly stop steroids
Patients who have been on corticosteroid therapy for more than a few weeks develop HPA axis suppression. Abrupt discontinuation, even of prescribed therapeutic steroids for other conditions, can precipitate adrenal crisis. Any steroid dose reduction must be gradual and supervised by a provider.
Emergency injection kit education
- Store the kit at home, at work, and while traveling
- Keep a kit in a temperature-stable location (do not leave in a hot car)
- Teach a family member or close friend to administer the injection if the patient becomes too ill to self-administer
- Practice technique with a training device or with the provider team before discharge
- Check expiration dates at every provider visit and refill proactively
Exercise and activity
- Patients can exercise normally with stable disease on replacement therapy
- Strenuous exercise is a recognized crisis trigger (2024 ESE/ES guideline); ask the endocrinology team whether an extra dose is advised before prolonged or high-intensity activity
- Teach the patient to recognize early hypoglycemia symptoms during exercise and carry fast-acting glucose
Dietary guidance
- No strict dietary restrictions in well-managed Addison’s disease
- Do not restrict salt (Endocrine Society 2016 rec 3.7); liberal salt intake helps (aldosterone deficiency increases sodium needs, especially in hot weather, during heavy exercise, or with significant sweating)
- Adequate carbohydrate intake to reduce hypoglycemia risk
- Patients on fludrocortisone who develop edema or hypertension may need dietary sodium reviewed with provider (possible dose adjustment)
Addison’s disease vs Cushing’s syndrome: comparison table
These two conditions represent opposite ends of the cortisol spectrum. Cushing’s syndrome is cortisol excess; Addison’s disease is cortisol deficiency. They are the two most commonly paired NCLEX endocrine questions. This table consolidates the key differentiators.
| Feature | Addison's disease (cortisol deficiency) | Cushing's syndrome (cortisol excess) |
|---|---|---|
| Cortisol level | LOW | HIGH |
| ACTH (primary) | HIGH (pituitary drives compensation) | HIGH if pituitary adenoma (Cushing's disease); LOW if adrenal tumor or exogenous steroids |
| Weight | Weight LOSS, anorexia | Weight GAIN, central obesity, moon face, buffalo hump |
| Blood pressure | HYPOTENSION, orthostatic changes | HYPERTENSION (cortisol has permissive vascular effects) |
| Blood glucose | HYPOGLYCEMIA (cortisol promotes gluconeogenesis) | HYPERGLYCEMIA (cortisol causes insulin resistance) |
| Sodium | HYPONATREMIA (aldosterone deficiency) | Usually normal or only marginally elevated – cortisol has mineralocorticoid activity at high levels, but water is retained along with sodium, so serum concentration changes little |
| Potassium | HYPERKALEMIA (aldosterone deficiency) | HYPOKALEMIA when present (cortisol drives K+ loss), often with metabolic alkalosis – but this occurs in only about a third of classic Cushing's cases and is most characteristic of ectopic ACTH syndrome |
| Skin | Hyperpigmentation (excess ACTH → MSH-like activity) | Thin skin, easy bruising, purple striae, acne |
| Muscle | Weakness, fatigue (cortisol drives muscle energy) | Proximal muscle WASTING (catabolism), weakness |
| Immune function | Possible eosinophilia, lymphocytosis | Immunosuppression – increased infection risk |
| Bones | Not directly affected acutely | Osteoporosis (cortisol inhibits osteoblasts) |
| Mood | Depression, apathy, irritability | Euphoria, emotional lability, depression, psychosis |
| Aldosterone | LOW (adrenal cortex destroyed) | Normal to slightly elevated |
| Diagnostic test | ACTH stimulation test (cosyntropin) – confirm cortisol fails to rise | 24-h urine free cortisol, late-night salivary cortisol, 1-mg overnight dexamethasone suppression test |
| Treatment direction | REPLACE cortisol (hydrocortisone) + mineralocorticoid (fludrocortisone) | REMOVE or BLOCK cortisol source (surgery, adrenolytic agents) |
| Emergency | Addisonian crisis – severe hypotension, treat with hydrocortisone 100 mg IV + NS | Post-adrenalectomy adrenal crisis – same treatment |
For a full discussion of Cushing’s syndrome pathophysiology, diagnostic workup, and nursing care, see the Cushing’s syndrome nursing reference.
NCLEX-style practice questions
Question 1
A nurse is caring for a patient newly diagnosed with Addison’s disease. During skin assessment, which finding does the nurse expect that is most characteristic of primary (not secondary) adrenal insufficiency?
A. Thin, translucent skin with easy bruising B. Grayish-brown discoloration of the buccal mucosa C. Periorbital edema and pretibial myxedema D. Yellowing of the sclerae and palms
Correct answer: B
Rationale: New hyperpigmentation of the buccal mucosa is a classic sign of primary adrenal insufficiency. In primary Addison’s disease, the adrenal cortex is destroyed, removing negative feedback on the pituitary. The pituitary increases ACTH output dramatically. ACTH and melanocyte-stimulating hormone (MSH) share the same POMC precursor and both activate melanocortin-1 receptors (MC1R) on melanocytes, causing increased melanin production. This produces bronze-to-brown discoloration at sun-exposed areas, pressure points, skin folds, and most distinctively, the buccal mucosa and gingiva. Secondary adrenal insufficiency involves ACTH deficiency – no hyperpigmentation occurs. Option A describes findings in Cushing’s syndrome. Option C describes hypothyroid myxedema. Option D suggests jaundice, unrelated to adrenal function.
Question 2
A patient with known Addison’s disease is admitted with severe hypotension (BP 78/42 mmHg), vomiting, confusion, and a temperature of 39.8°C. The patient reports having vomiting for 12 hours and was unable to take their morning hydrocortisone. Which action is the nurse’s highest priority?
A. Obtain a serum cortisol level before initiating any treatment B. Administer hydrocortisone 100 mg IV bolus as ordered C. Initiate a 12-lead ECG to rule out MI D. Administer fludrocortisone 0.1 mg orally
Correct answer: B
Rationale: This patient is in adrenal crisis – a life-threatening emergency characterized by severe hypotension, vomiting, altered mental status, and fever in the context of missed steroid doses. The immediate priority is IV hydrocortisone 100 mg bolus. While it is ideal to draw labs before giving steroids, this must not delay treatment in a hemodynamically unstable patient. The nurse should draw labs (cortisol, ACTH, BMP) during IV placement – but the hydrocortisone bolus cannot wait. Option A is incorrect because waiting for a cortisol result in a crashing patient could be fatal. Option C is not the immediate priority given the clear clinical picture of adrenal crisis. Option D is incorrect because oral fludrocortisone is ineffective in crisis (patient is vomiting, unable to absorb oral medications, and fludrocortisone does not address glucocorticoid deficiency).
Question 3
A nurse is educating a patient with Addison’s disease about sick day management. Which statement by the patient indicates a need for further teaching?
A. “I will double my hydrocortisone dose if I develop a fever.” B. “If I start vomiting and can’t keep my pills down, I’ll use my injection kit and go to the ER.” C. “I wear my medical alert bracelet every day in case I’m in an accident.” D. “If I feel a little tired, I’ll skip my afternoon hydrocortisone dose to avoid over-replacement.”
Correct answer: D
Rationale: Skipping steroid doses based on symptom perception is extremely dangerous. Patients with Addison’s disease have no functional adrenal reserve – fatigue may reflect under-replacement (a signal to notify the provider) or normal fluctuation, but skipping doses risks precipitating adrenal crisis. Cortisol replacement must be taken consistently. Option A is correct – doubling the dose for febrile illness is appropriate sick day management. Option B is correct – vomiting that prevents oral absorption requires immediate injection and emergency evaluation; this is the exact scenario in which patients die without recognizing the urgency. Option C is correct and essential – the medical alert bracelet is non-negotiable for unconscious emergency situations.
Question 4
A nurse reviews morning lab results for a patient with suspected adrenal insufficiency. Which combination of findings is most consistent with primary Addison’s disease?
A. Low cortisol, low ACTH, hyponatremia, hypokalemia B. High cortisol, high ACTH, hypernatremia, hypokalemia C. Low cortisol, HIGH ACTH, hyponatremia, hyperkalemia D. Low cortisol, low ACTH, hypernatremia, hyperkalemia
Correct answer: C
Rationale: In primary adrenal insufficiency, the adrenal cortex is destroyed. Cortisol falls, removing negative feedback on the pituitary – ACTH rises well above the reference range (the Endocrine Society uses more than twice the upper limit). Aldosterone deficiency causes sodium wasting (hyponatremia) and potassium retention (hyperkalemia). This combination – low cortisol + HIGH ACTH + hyponatremia + hyperkalemia – is the classic electrolyte and hormonal pattern of primary Addison’s disease. Option A describes secondary/tertiary adrenal insufficiency (low ACTH, and hypokalemia is not expected). Option B describes Cushing’s disease (excess cortisol and ACTH). Option D cannot occur: low ACTH with hypernatremia and hyperkalemia is not consistent with any single adrenal disorder pattern.
Question 5
A patient with Addison’s disease is scheduled for an elective appendectomy. The patient asks the nurse why their hydrocortisone dose will be increased around the surgery. Which response by the nurse is most accurate?
A. “Higher steroid doses prevent the surgical wound from becoming infected.” B. “Your body normally increases cortisol output during surgical stress, but your adrenal glands cannot do this – we replace that increase artificially.” C. “The anesthesia medications break down cortisol faster than usual, so you need a larger dose.” D. “The IV fluids used in surgery dilute your cortisol level, so we compensate by giving more.”
Correct answer: B
Rationale: A healthy adrenal gland increases cortisol output significantly during physiologic stress such as surgery (the cortisol stress response can rise several-fold above baseline). Patients with Addison’s disease have no functional adrenal reserve and cannot mount this response. Without supplemental hydrocortisone (stress dosing), the patient will develop adrenal crisis during or after surgery – severe hypotension refractory to vasopressors, hypoglycemia, and cardiovascular collapse. Stress dosing replaces the cortisol the patient’s own adrenal glands cannot produce. Option A is incorrect – glucocorticoids impair wound healing and immune function at high doses. Option C is clinically inaccurate. Option D is not a valid mechanism.
Question 6
The nurse is assessing a patient who has been treated for Addison’s disease for 5 years and is now on stable doses of hydrocortisone and fludrocortisone. The patient reports increasing fatigue, dizziness, salt cravings, and nausea over the past 3 weeks. Blood pressure is 94/58 mmHg. The nurse suspects which problem?
A. Over-replacement of fludrocortisone causing volume overload B. Under-replacement – inadequate steroid dosing relative to current needs C. Hyperglycemia from excess hydrocortisone D. Cushing’s syndrome from long-term steroid use
Correct answer: B
Rationale: The symptoms described – fatigue, dizziness, salt craving, nausea, and hypotension – are classic features of adrenal insufficiency and suggest the current replacement regimen is inadequate. This can occur because the patient’s needs have increased (illness, physiologic stress, life change), adherence has declined, or dosing simply needs adjustment over time. The priority nursing action is to notify the provider and review the current regimen. Option A is incorrect – over-replacement with fludrocortisone would cause hypertension, edema, and hypokalemia, the opposite of the findings. Option C is incorrect – hyperglycemia from excess hydrocortisone would present with polyuria, polydipsia, and elevated glucose. Option D describes findings of cortisol excess (moon face, central obesity, purple striae), which are not present.
Summary: high-yield NCLEX takeaways
- Hyperpigmentation = primary Addison’s = excess ACTH. No hyperpigmentation in secondary or tertiary adrenal insufficiency (ACTH is low in those).
- Lab pattern: low cortisol + HIGH ACTH + hyponatremia + hyperkalemia + hypoglycemia.
- Gold standard diagnosis: ACTH (cosyntropin) stimulation test – a peak cortisol below 18 mcg/dL (500 nmol/L, assay dependent) at 30 or 60 minutes indicates insufficiency.
- Adrenal crisis = emergency: hydrocortisone 100 mg IV or IM, then 200 mg/24 h, + 1 L isotonic saline in the first hour + treat the trigger.
- Stress dosing is mandatory – illness with fever means doubling the daily dose; surgery needs IV hydrocortisone; vomiting = inject + go to ER.
- Medical alert bracelet is non-negotiable – always on, contains diagnosis and emergency treatment.
- Fludrocortisone = mineralocorticoid, primary Addison’s only – secondary adrenal insufficiency does not require it.
- Hydrocortisone timing mirrors diurnal rhythm – largest dose on waking to peak with natural cortisol.
- Darkening of old scars = classic NCLEX clue for rising ACTH in primary Addison’s.
- Compare with Cushing’s: Addison’s = low BP, low glucose, low Na+, high K+, weight loss, hyperpigmentation. Cushing’s = high BP, high glucose, sodium normal to marginally high, K+ low when affected, weight gain, thin/fragile skin.
For further reading on related endocrine disorders, see the hypothyroidism nursing reference, hyperthyroidism nursing reference, Cushing’s syndrome nursing reference, and diabetes mellitus nursing reference. For electrolyte management in adrenal crisis, see the electrolyte imbalances reference and the nursing lab values cheat sheet.
References
- Bornstein SR, Allolio B, Arlt W, et al. Diagnosis and treatment of primary adrenal insufficiency: an Endocrine Society clinical practice guideline. Journal of Clinical Endocrinology & Metabolism. 2016;101(2):364–389. doi:10.1210/jc.2015-1710
- National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). Adrenal Insufficiency & Addison’s Disease. National Institutes of Health; last reviewed September 2018. Available at: https://www.niddk.nih.gov/health-information/endocrine-diseases/adrenal-insufficiency-addisons-disease
- Charmandari E, Nicolaides NC, Chrousos GP. Adrenal insufficiency. The Lancet. 2014;383(9935):2152–2167. doi:10.1016/S0140-6736(13)61684-0
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- Pfizer. Solu-Cortef (hydrocortisone sodium succinate for injection) prescribing information. Revised July 2024. DailyMed setid 65eefd58-b166-4d71-ade6-45c8fdf86922.
- Fludrocortisone acetate tablets, USP 0.1 mg, prescribing information. DailyMed setid 933e28ef-05e8-419e-a786-21eab67f33a2 (published July 2026).