What is the treatment approach for adrenal hyperplasia?

Treatment Approach for Adrenal Hyperplasia

The treatment of adrenal hyperplasia depends on the specific type, with surgical intervention (laparoscopic adrenalectomy) recommended for unilateral adenomas and medical management with mineralocorticoid antagonists or hormone replacement for bilateral hyperplasia. 1

Types of Adrenal Hyperplasia and Their Management

Primary Aldosteronism (Hyperaldosteronism)

• Diagnostic approach includes measuring plasma aldosterone and renin activity, with a plasma aldosterone-to-renin ratio >30 suggesting primary hyperaldosteronism 1
• Confirmatory testing with saline suppression test or salt loading test is recommended due to potential false positives/negatives 1
• Adrenal vein sampling is considered the gold standard for distinguishing between unilateral adenoma and bilateral hyperplasia in surgical candidates 1
• For unilateral adenoma: Laparoscopic adrenalectomy is the treatment of choice 1
• For bilateral adrenal hyperplasia: Medical management with spironolactone or eplerenone is recommended to control hypertension and hypokalemia 1
• In patients younger than 40 years with imaging showing only one affected gland, adrenal vein sampling may be excluded as bilateral hyperplasia is rare in this population 1

Cushing Syndrome (Hypercortisolism)

• For ACTH-independent Cushing syndrome caused by bilateral multinodal hyperplasia, adrenal vein sampling of cortisol production guides treatment 1
• If cortisol production is asymmetric: Laparoscopic unilateral adrenalectomy with removal of the most active side is recommended, with postoperative corticosteroid supplementation 1
• If cortisol production is symmetric: Medical management is indicated 1
• Medical management of hypercortisolism includes adrenostatic agents such as ketoconazole (400-1200 mg/day) and mitotane 1
• Octreotide can be considered for ectopic Cushing syndrome if the tumor is Octreoscan-positive 1

Congenital Adrenal Hyperplasia (CAH)

• The main goal is to replace deficient hormones and suppress excess androgen production 2, 3
• Glucocorticoid replacement: Hydrocortisone is the drug of choice at 15-25 mg daily in split doses for adults 1, 4
• For children: Hydrocortisone dosing should be 6-10 mg/m² of body surface area 1, 4
• Mineralocorticoid replacement: Most patients with primary adrenal insufficiency should take 50-200 μg fludrocortisone as a single daily dose 1
• Children and younger adults may require higher doses of fludrocortisone 1
• Patients should be advised to take salt and salty foods ad libitum and avoid licorice and grapefruit juice 1
• Regular monitoring is essential to prevent complications such as metabolic syndrome, cardiovascular morbidity, and osteoporosis 3, 5

Management of Adrenal Crisis

• Adrenal crisis should be treated immediately with IV or IM hydrocortisone, 100 mg followed by 100 mg every 6-8 hours until recovered 1
• Isotonic (0.9%) sodium chloride solution should be administered at an initial rate of 1 L/hour until hemodynamic improvement 1
• The underlying precipitant of adrenal crisis (e.g., infection) should be identified and treated 1
• Mineralocorticoid replacement with fludrocortisone should be restarted when the hydrocortisone dose falls to <50 mg/day 1

Follow-up and Monitoring

• Patients with adrenal hyperplasia should be reviewed at least annually 1
• Assessment should include health and well-being, weight, blood pressure, and serum electrolytes 1
• Monitoring for the development of new autoimmune disorders, particularly hypothyroidism, is recommended 1
• Assessment for complications of glucocorticoid therapy should include monitoring of bone mineral density every 3-5 years 1
• For patients with congenital adrenal hyperplasia, transition from pediatric to adult care requires regular follow-up by a multidisciplinary team 3, 6

Common Pitfalls and Caveats

• CT imaging alone is not always reliable for distinguishing between unilateral adenoma and bilateral hyperplasia, with reports suggesting 25% of patients would have had unnecessary adrenalectomy based on CT findings alone 1
• Both under-treatment and over-treatment of CAH can be harmful to long-term cardiovascular health 5
• Postoperative corticosteroid supplementation is required after adrenalectomy until recovery of the hypothalamus-pituitary-adrenal axis 1
• Control of CAH can be difficult during puberty due to alterations in hydrocortisone clearance, requiring adjustment of dosing schedules 4
• All patients with adrenal insufficiency should wear medical alert identification and carry a steroid alert card 1