What is the treatment for panhypopituitarism?

Treatment of Panhypopituitarism

Treatment of panhypopituitarism requires comprehensive hormone replacement therapy with corticosteroids, thyroid hormone, sex hormones, and potentially growth hormone, with corticosteroid replacement always initiated before thyroid hormone to prevent adrenal crisis. 1

Hormone Replacement Therapy

1. Corticosteroid Replacement (First Priority)

• Initial dosing:
  • Hydrocortisone 15-20 mg daily in divided doses (typically 10-15 mg in morning, 5 mg in afternoon) 1
  • Alternative: Prednisone 5 mg daily (equivalent to hydrocortisone 20 mg) for patients who cannot adhere to multiple daily dosing 1
• Stress dosing:
  • Minor illness: Double or triple daily dose
  • Major illness/surgery: Hydrocortisone 100 mg IV before surgery, then 50-100 mg every 6-8 hours, tapering to maintenance over 5-7 days 1
  • Adrenal crisis: Immediate treatment with IV or IM hydrocortisone 100 mg followed by 100 mg every 6-8 hours until recovered, plus isotonic saline solution at initial rate of 1 L/hour until hemodynamic improvement 2

2. Thyroid Hormone Replacement

• Important: Never start thyroid hormone before corticosteroids - can precipitate adrenal crisis 1
• Levothyroxine (T4) dosing based on weight and age 1
  • Typical starting dose: 1.6 μg/kg/day in adults
  • Goal: Free T4 in upper half of reference range (don't rely on TSH for monitoring) 1

3. Sex Hormone Replacement

• Males:
  • Testosterone replacement via injections, gels, or patches 1
• Females:
  • Estrogen with progestogen (if uterus present) or estrogen-only (if hysterectomized)
  • Transdermal 17β-estradiol (50-100 μg daily) preferred due to better safety profile 1

4. Growth Hormone Replacement

• Consider in adults with confirmed GH deficiency
• Benefits: Improved body composition, exercise capacity, lipid profile, and quality of life 1

5. Other Hormone Replacements

• Desmopressin for diabetes insipidus if present (posterior pituitary dysfunction) 1
• DHEA supplementation may be considered in women with low libido/energy 1

Patient Education and Monitoring

Essential Patient Education

• All patients should:
  • Wear medical alert identification (bracelet/necklace) 2, 1
  • Carry a steroid alert card 2
  • Receive education on managing daily medications and concurrent illnesses 2
  • Have supplies for self-injection of parenteral hydrocortisone 2, 1

Monitoring

• Annual review with assessment of:
  • Health and well-being
  • Weight and blood pressure
  • Serum electrolytes 2
  • Monitoring for development of new autoimmune disorders, particularly hypothyroidism 2
  • Bone mineral density every 3-5 years 2
  • Free T4 levels (not TSH) 1
  • IGF-1 for growth hormone adequacy 1
  • Sex hormone levels 1

Special Considerations

Perioperative Management

• High risk for hemodynamic instability during surgical stress 3
• Stress-dose steroids essential before and during surgery 1, 3
• Continue thyroid replacement throughout perioperative period 1

Adrenal Crisis Prevention

• Inadequate stress dosing is a common cause of adrenal crisis 1
• Patients should be advised to take salt and salty foods ad libitum 2
• Avoid licorice and grapefruit juice 2

Underlying Etiology

• Common causes requiring specific management include pituitary adenomas, empty sella syndrome, and immune checkpoint inhibitor therapy 1, 4, 5
• In cases of pituitary adenoma, surgical removal by trans-sphenoidal approach may be preferable 4
• Consider radiotherapy for patients with uncontrolled tumor growth and incomplete surgical and medical response 2

Pitfalls and Caveats

• Progressive loss of anterior pituitary function can occur over time, requiring ongoing reassessment 6
• Pancytopenia can result from hormonal deficiencies in panhypopituitarism 7
• Atypical presentations including syncope and hypotension may be the first signs of panhypopituitarism 5
• Patients with panhypopituitarism are at increased risk of mortality, primarily due to cardiovascular, infectious, and malignant diseases 1