What is the management algorithm for adrenal insufficiency?

Management Algorithm for Adrenal Insufficiency

The management of adrenal insufficiency requires a systematic approach based on disease severity, with immediate treatment for suspected adrenal crisis and appropriate maintenance therapy for long-term management. 1

Diagnosis

• Consider adrenal insufficiency in patients with unexplained collapse, hypotension, vomiting, diarrhea, hyperpigmentation, hyponatremia, hyperkalaemia, acidosis, or hypoglycemia 1
• Diagnostic workup includes:
  • Morning ACTH and cortisol levels (primary AI: low cortisol, high ACTH; secondary AI: low cortisol, low ACTH) 1, 2
  • Basic metabolic panel to assess for electrolyte abnormalities 1, 2
  • ACTH stimulation test for equivocal cases (peak cortisol <500 nmol/L is diagnostic) 1
• Never delay treatment of suspected acute adrenal insufficiency for diagnostic procedures 1

Treatment Algorithm by Severity

Adrenal Crisis (Severe Symptoms, Life-Threatening)

• Immediate IV or IM hydrocortisone 100 mg, followed by 100 mg every 6-8 hours until recovered 1
• IV isotonic (0.9%) sodium chloride solution at an initial rate of 1 L/hour until hemodynamic improvement 1
• Identify and treat underlying precipitant (e.g., infection) 1
• Taper stress-dose corticosteroids to maintenance doses over 7-14 days after discharge 1

Moderate Symptoms (Able to Perform ADLs)

• Outpatient treatment at 2-3 times maintenance dose (prednisone 20 mg daily or hydrocortisone 20-30 mg morning, 10-20 mg afternoon) 1
• Taper stress-dose corticosteroids to maintenance doses over 5-10 days 1
• Consider endocrine consultation 1

Mild Symptoms or Maintenance Therapy

• Primary adrenal insufficiency:
  • Hydrocortisone 15-25 mg daily in split doses (first dose upon waking, last dose not <6 hours before bedtime) 1
  • Fludrocortisone 50-200 μg as a single daily dose 1, 3
  • Salt and salty foods ad libitum 1
• Secondary adrenal insufficiency:
  • Hydrocortisone 10-20 mg morning, 5-10 mg afternoon 2, 4
  • No fludrocortisone required 2, 4

Special Considerations

Primary vs. Secondary Adrenal Insufficiency

• Primary AI (adrenal gland failure): requires both glucocorticoid and mineralocorticoid replacement 1
• Secondary AI (pituitary dysfunction): requires only glucocorticoid replacement 1, 2
• When multiple hormone deficiencies are present, always start corticosteroids several days before thyroid hormone to prevent precipitating adrenal crisis 2, 4

Patient Education and Follow-up

• All patients should:
  • Wear medical alert identification and carry a steroid alert card 1
  • Receive education on stress dosing (doubling or tripling doses during illness) 1
  • Have supplies for self-injection of parenteral hydrocortisone 1
• Annual follow-up with assessment of:
  • Health and well-being
  • Weight and blood pressure
  • Serum electrolytes
  • Development of new autoimmune disorders (particularly hypothyroidism) 1
• Bone mineral density monitoring every 3-5 years 1

Stress Dosing Guidelines

• Minor illness (fever <38°C): Double usual daily dose 4, 5
• Moderate illness (fever >38°C, vomiting, diarrhea): Triple usual daily dose 4, 5
• Severe illness or trauma: IV hydrocortisone 100 mg immediately, then 100 mg every 6-8 hours 1
• Surgery: IV hydrocortisone 100 mg before induction, followed by continuous infusion or divided doses during and after procedure 1

Common Pitfalls and Caveats

• Failure to recognize adrenal crisis as a medical emergency requiring immediate treatment 1
• Inadequate stress dosing during illness or procedures 1
• Overlooking mineralocorticoid replacement in primary adrenal insufficiency 1, 3
• Initiating thyroid replacement before adequate glucocorticoid replacement 2, 4
• Missing iatrogenic adrenal insufficiency in patients on chronic glucocorticoid therapy 6, 7

By following this algorithm, clinicians can effectively manage adrenal insufficiency to reduce morbidity and mortality while optimizing quality of life for affected patients.