Management of Symptomatic Primary Adrenal Insufficiency in Carney Complex

Initial Assessment and Optimization Strategy

The American College of Endocrinology suggests that patients with primary adrenal insufficiency require optimization of their current hormone replacement therapy, specifically the addition of DHEA supplementation to address symptoms of decreased libido and poor sleep, after first ensuring adequate glucocorticoid and mineralocorticoid replacement 1
The Endocrine Society recommends assessing for under-replacement signs, such as morning fatigue, salt cravings, lightheadedness, and whether symptoms improve after taking hydrocortisone, to verify adequate glucocorticoid replacement 3
Standard hydrocortisone replacement is 15-25 mg daily, typically divided as 10 mg + 5 mg + 2.5 mg (at 07:00, 12:00, and 16:00), according to the American Association of Clinical Endocrinologists 4
The American College of Endocrinology advises checking for drug interactions, such as NSAIDs, which should be avoided as they interact with fludrocortisone and may worsen joint symptoms 3, 5

The Endocrine Society suggests assessing for salt cravings, orthostatic hypotension, and peripheral edema to evaluate mineralocorticoid adequacy 2
Standard dosing for fludrocortisone is 50-200 µg daily, with younger adults sometimes requiring up to 500 µg daily, according to the American Association of Clinical Endocrinologists 1, 6

The American College of Endocrinology recommends initiating a 6-month trial of DHEA 25 mg once daily in the morning to address adrenal androgen deficiency 1, 7
Baseline laboratory testing should include measuring morning serum DHEA sulfate, androstenedione, and testosterone levels, according to the Endocrine Society 1, 5
Target levels should be maintained in the normal range for premenopausal women, and monitoring strategy should include rechecking levels at 3 months and adjusting dose as needed 1, 8

The American College of Rheumatology advises avoiding NSAIDs, which interact with fludrocortisone and should be avoided, and considering glucocorticoid adequacy, as joint symptoms may reflect inadequate replacement during minor stressors 3, 5

The American College of Obstetricians and Gynecologists suggests that shortened menstrual periods may reflect androgen deficiency, and DHEA supplementation may normalize menstrual patterns 1
Monitoring for perimenopause is recommended, as early perimenopausal changes are possible and would be exacerbated by adrenal androgen deficiency 5

The American Association of Clinical Endocrinologists recommends educating patients on stress dosing to prevent adrenal crisis, including sick day rules, such as doubling oral glucocorticoid dose for 24-48 hours during acute illness 9

The Endocrine Society suggests reassessing symptoms after any adjustments to glucocorticoid or mineralocorticoid dosing at 2-4 weeks, and checking morning DHEAS, androstenedione, and testosterone levels at 3 months 1
Deciding whether to continue DHEA based on clinical efficacy should be done at 6 months, according to the American College of Endocrinology 1, 5