Treatment of Abnormal Cortisol Levels
The treatment of abnormal cortisol levels depends on whether levels are high (hypercortisolism/Cushing syndrome) or low (adrenal insufficiency), with surgical intervention being first-line for most cases of hypercortisolism and hormone replacement therapy being essential for adrenal insufficiency. 1
Treatment of Hypercortisolism (Cushing Syndrome)
Surgical Approaches
- Transsphenoidal surgery is the first-line treatment for pituitary tumors causing Cushing's disease 1
- For ectopic ACTH-producing tumors, surgical removal of the primary tumor should be pursued when possible 1
- Bilateral laparoscopic adrenalectomy is recommended for patients with unresectable primary tumors 1
Medical Management
- Adrenostatic agents like ketoconazole (400-1200 mg/day) are treatment options when surgery is not possible or has failed 1
- Mitotane is particularly useful for adrenal carcinoma causing hypercortisolism 1
- Octreotide can be considered for ectopic Cushing syndrome if the tumor is Octreoscan-positive 1
Treatment of Adrenal Insufficiency (Low Cortisol)
Glucocorticoid Replacement
- Hydrocortisone 15-25 mg daily in divided doses is the standard treatment for primary adrenal insufficiency 1, 2
- For secondary adrenal insufficiency, hydrocortisone 15-20 mg daily in divided doses is recommended 1
- Prednisone 3-5 mg daily can be used as an alternative to hydrocortisone 2
- Dosage must be individualized based on the specific disease entity and patient response 3
Mineralocorticoid Replacement
- Fludrocortisone (0.05-0.1 mg/day) is essential for primary adrenal insufficiency 1, 2
- Dosage should be adjusted based on volume status, sodium levels, and renin response 1
- Mineralocorticoid replacement is not needed for secondary adrenal insufficiency 2
Special Considerations and Management Pearls
Stress Dosing
- Increase glucocorticoid dose (typically 2-3 times maintenance dose) during illness or physical stress 1, 2
- For severe stress or adrenal crisis, injectable hydrocortisone (100 mg) should be administered 2
- Gradual dose reduction is essential when discontinuing therapy to prevent secondary adrenal insufficiency 3
Monitoring and Follow-up
- Regular follow-up of clinical symptoms and electrolytes is recommended for all patients 1
- For functioning tumors causing hypercortisolism, follow-up imaging and biomarkers every 3-6 months is advised 1
- Morning cortisol levels should be monitored to assess adequacy of replacement therapy 2
Patient Education and Safety
- Medical alert bracelet or necklace is essential for patients with adrenal insufficiency 1
- Education on steroid stress dosing and emergency injections is crucial 1, 2
- Patients on immunosuppressant doses of corticosteroids should avoid exposure to chickenpox or measles 3
Adrenal Crisis Management
- Adrenal crisis is life-threatening and requires immediate treatment with IV hydrocortisone and fluid resuscitation 1, 2
- All patients with adrenal insufficiency should have an emergency kit with injectable glucocorticoids 2
- Failure to recognize and treat adrenal crisis promptly can be fatal 1
Pitfalls and Caveats
- Drug-induced secondary adrenal insufficiency may occur with abrupt discontinuation of glucocorticoid therapy 3
- Corticosteroids should be used cautiously in patients with infections, osteoporosis, hypertension, and psychiatric disorders 3
- Growth and development should be carefully monitored in children on prolonged corticosteroid therapy 3
- Cortisol levels during "low-dose" hydrocortisone therapy can initially exceed physiological levels significantly 4