What is the treatment for adrenal insufficiency due to partially empty sella?

Treatment of Adrenal Insufficiency Due to Partially Empty Sella

Adrenal insufficiency due to partially empty sella requires hormone replacement therapy with glucocorticoids (hydrocortisone) as the primary treatment, with careful dose adjustment based on clinical response. 1

Diagnosis Confirmation

Before initiating treatment, confirm the diagnosis with:

• Morning cortisol and ACTH levels
• ACTH stimulation test (1 mcg cosyntropin test)
• MRI of the pituitary to confirm partially empty sella

Treatment Algorithm

1. Initial Hormone Replacement

• Glucocorticoid replacement:

  • Hydrocortisone 15-25 mg daily in divided doses (typically 10-15 mg in morning, 5-10 mg in afternoon) 1
  • Divided dosing mimics physiological cortisol secretion pattern
  • Alternative: Prednisone 5 mg in morning and 2.5 mg in afternoon

• Mineralocorticoid replacement:

  • Not typically required in secondary adrenal insufficiency (partially empty sella)
  • Fludrocortisone is only indicated for primary adrenocortical insufficiency (Addison's disease) 2

2. Dose Adjustment

• Adjust based on clinical symptoms (fatigue, appetite, weight, blood pressure)
• Avoid over-replacement which can lead to cushingoid features
• Under-replacement may result in persistent fatigue, hypotension, and hyponatremia

3. Stress Dosing

During periods of illness, injury, or stress:

• Minor illness (fever, cold):

  • Double oral hydrocortisone dose for duration of illness 1

• Moderate stress (non-severe infection, minor procedures):

  • Triple oral dose for 24-48 hours 1

• Severe stress (surgery, severe illness, trauma):

  • Hydrocortisone 100 mg IV bolus followed by 100-300 mg/day as continuous infusion or divided IV/IM doses every 6 hours
  • IV fluids (isotonic saline) 1

4. Adrenal Crisis Management

For adrenal crisis (severe hypotension, vomiting, confusion):

• Immediate IV hydrocortisone 100 mg bolus
• Rapid IV isotonic saline (1 L over first hour)
• Continue IV hydrocortisone 100-300 mg/day in divided doses
• Taper to oral replacement over 1-3 days as condition improves 1

Patient Education and Monitoring

Patient Education

• Provide emergency hydrocortisone injection kit
• Teach stress dosing rules
• Medical alert bracelet/card identifying adrenal insufficiency
• Instructions on when to seek medical help

Monitoring

• Regular follow-up every 3-6 months
• Annual comprehensive evaluation including:
  • Clinical assessment (weight, blood pressure, symptoms)
  • Electrolytes (sodium, potassium)
  • Screen for other pituitary hormone deficiencies 1

Special Considerations

• Hyponatremia management: Common in secondary adrenal insufficiency, requires careful correction to avoid osmotic demyelination syndrome 3
• Other pituitary hormones: Assess for other hormone deficiencies (thyroid, gonadal) that may accompany partially empty sella 4, 5
• Avoid abrupt discontinuation: Never stop glucocorticoid therapy abruptly

Common Pitfalls

1. Failure to provide stress dosing education: Patients must understand when and how to increase their dose during illness or stress
2. Over-replacement: Excessive glucocorticoid doses can lead to iatrogenic Cushing's syndrome, osteoporosis, and metabolic complications 6
3. Under-replacement: Insufficient dosing can lead to persistent fatigue, hypotension, and risk of adrenal crisis
4. Missing concomitant thyroid deficiency: Always check thyroid function, as hypothyroidism often coexists with ACTH deficiency 1
5. Inappropriate mineralocorticoid use: Fludrocortisone is typically not needed in secondary adrenal insufficiency as the renin-angiotensin-aldosterone system remains intact

By following this treatment approach, patients with adrenal insufficiency due to partially empty sella can achieve normal quality of life and avoid the morbidity and mortality associated with adrenal crisis.