Sheehan's syndrome medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Iqra Qamar M.D.[2].

Treatment involves appropriate hormone replacement therapy that results in complete recovery and reversal of symptoms.

• Treatment involves appropriate hormone replacement therapy, which must be taken for the rest of your life that results in significant improvement and reversal of not only the physical symptoms, but also the psychological symptoms.^{[1][2][3][4][5][6][7][8][9][10][11][12][13][14]}

• Preferred regimen: Hydrocortisone 100 mg IV bolus, then 300 mg/day IV divided q8hr or continuous infusion for 48 hours
  • Once patient is stable: 50 mg PO q8hr for 6 doses, later on tapered to 30-50 mg/day PO in divided doses

Chronic setting

• Preferred regimen: Hydrocortisone 15-25 mg/day PO divided q8-12hr

NOTE

• Dosage is increased in patients with severe deficiency, increased body weight and in times of surgery, illness, procedures, and other stresses.
• Unfortunately, there is no established test to assess adequate hormonal replacement. Plasma ACTH measurement and serum/salivary/urinary cortisol values are all unreliable. So assessment of adequate hormonal replacement is based on clinical basis with Cushingoid features showing excessive replacement while symptoms of adrenal insufficiency suggesting insufficient hormonal replacement.
• Glucocorticoid replacement can cause polyuria due to unmasking of underlying central diabetes insipidus. DDAVP is the treatment of choice for patients with DI.^[15]
• Mineralocorticoids are rarely required as aldosterone secretion is primarily regulated by angiotensin II and potassium not by ACTH.

• Levothyroxine 1.7 mcg/kg qDay or 100-125 mcg PO qDay

• Levothyroxine 12.5-25 mcg PO qDay and later on dose can be adjusted by 25 mcg/day q2-4 Week PRN

• In patients with combined hypothyroidism and hypocortisolism, glucocorticoids (physiologic doses and increased doses in stress) are replaced before thyroid hormone replacement, because treating the hypothyroidism alone by levothyroxine can worsen the severity of cortisol deficiency by increasing the clearance of cortisol. So, it is important to assess adrenal function, including corticotropin (ACTH) reserve, before administering T4 (levothyroxine).
• American Thyroid Association (ATA) recommends dose adjustment to keep serum free T4 concentration in upper half of reference range.

• If fertility required:
  • Such women are offered ovulation induction. Pregnancy can be made possible by giving exogenous gonadotropins or pulsatile GnRH.
  • Women with GnRH deficiency can be offered either pulsatile GnRH or gonadotropin therapy.
  • Women with gonadotropin deficiency are given gonadotropins only.

• If fertility not required:
  • Such women are treated with estrogen-progestin replacement therapy by using the traditional regimen of estradiol on days 1 through 25 of each month and progesterone on days 16 through 25 of each month.
  • Another regimen includes continuous transdermal estradiol throughout the month, with progestin added days 1 to 10 of the calendar month.

• Androgens can be given to females having low libido.^[16]

• GH is replaced on case to case basis starting with a low dose (0.1-0.3 mg/day) and titrated upwards by 0.1 mg/d/month with repeated measurement of hormone levels every month, initially for the first 6 months followed by yearly measurements; replaced once all other hormones have been replaced.^[17]

• A study was done on 5 women with prolactin deficiency caused by Sheehan’s syndrome or other causes that showed increased milk production upon subcutaneous administration of r-hPRL (recombinant human prolactin) every 12 hours for 28 days.^[18]

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