What happens in adrenal insufficiency?

From the Guidelines

Adrenal insufficiency is a life-threatening condition that requires immediate treatment with injectable hydrocortisone (100 mg) in cases of adrenal crisis, which can be triggered by stress, illness, or injury. This condition occurs when the adrenal glands fail to produce adequate amounts of essential hormones, primarily cortisol and sometimes aldosterone. It can be primary (Addison's disease), where the adrenal glands themselves are damaged, or secondary, resulting from pituitary gland dysfunction that reduces ACTH production. Symptoms include fatigue, weakness, weight loss, decreased appetite, abdominal pain, low blood pressure, dizziness, and darkening of the skin in primary cases.

Treatment and Management

Treatment typically involves hormone replacement therapy with glucocorticoids like hydrocortisone (15-25 mg daily in divided doses), prednisone (3-5 mg daily), or dexamethasone (0.25-0.75 mg daily) 1. For primary adrenal insufficiency, mineralocorticoid replacement with fludrocortisone (0.05-0.2 mg daily) is also necessary. Patients must carry emergency medication and wear medical alert identification, as adrenal crisis requires immediate treatment. Regular monitoring of hormone levels and dose adjustments are essential for proper management, as evidenced by guidelines for the management of glucocorticoids during the peri-operative period for patients with adrenal insufficiency 1.

Risk of Adrenal Crisis

The risk of adrenal crisis is significant, especially in patients with primary or secondary adrenal insufficiency who are taking physiological replacement doses of corticosteroids, as well as those on long-term oral corticosteroid therapy 1. It is crucial for these patients to receive stress doses of hydrocortisone during the peri-operative period to prevent adrenal crisis. The prevalence of adrenal insufficiency in adult and pediatric populations is notable, with approximately seven in 1000 people prescribed long-term oral corticosteroid therapy, creating a large population at risk of adrenal crisis 1.

Key Recommendations

• Hydrocortisone 100 mg intravenously at the start of surgery, followed by an infusion of 200 mg/24h is recommended for patients with adrenal insufficiency undergoing surgery 1.
• Patients should double their regular oral replacement dose of hydrocortisone for 48 hours after surgery, and for up to a week following major surgery before resuming their maintenance dose 1.
• Endocrine consultation prior to surgery or any procedure is essential for stress-dose planning and to ensure that patients with adrenal insufficiency receive appropriate care 1.
• Patients must be educated on stress dosing and the importance of carrying a medical alert bracelet for adrenal insufficiency to trigger stress-dose corticosteroids by emergency medical services 1.

From the FDA Drug Label

Corticosteroids can produce reversible HPA axis suppression with the potential for glucocorticosteroid insufficiency after withdrawal of treatment Drug induced secondary adrenocortical insufficiency may be minimized by gradual reduction of dosage. This type of relative insufficiency may persist for months after discontinuation of therapy; therefore, in any situation of stress occurring during that period, hormone therapy should be reinstituted

In adrenal insufficiency, the body does not produce enough corticosteroids, such as hydrocortisone. This can occur due to HPA axis suppression caused by prolonged use of corticosteroids.

• Glucocorticosteroid insufficiency may occur after withdrawal of treatment.
• Adrenocortical insufficiency can persist for months after discontinuation of therapy.
• In situations of stress, hormone therapy should be reinstituted to minimize the risk of adrenal insufficiency 2

From the Research

Adrenal Insufficiency Overview

• Adrenal insufficiency is a life-threatening disorder characterized by the deficient production or action of glucocorticoids, with or without deficiency in mineralocorticoids and adrenal androgens 3.
• It can result from primary adrenal failure or secondary adrenal disease due to impairment of the hypothalamic-pituitary axis 3.

Clinical Manifestations

• The clinical manifestations of primary adrenal insufficiency include deficiency of all adrenocortical hormones and can also include signs of other concurrent autoimmune conditions 3.
• In secondary or tertiary adrenal insufficiency, the clinical picture results from glucocorticoid deficiency only, but manifestations of the primary pathological disorder can also be present 3.
• Common symptoms include fatigue, weight loss, gastrointestinal manifestations, and skin hyperpigmentation, which is specific to primary adrenal failure 4.

Diagnosis and Management

• Diagnosis can be challenging, especially in patients with secondary or tertiary adrenal insufficiency 3.
• Monitoring of substitutive treatment is well codified, and patient education is crucial in managing this chronic disease 4.
• Treatment typically involves glucocorticoid replacement with hydrocortisone and mineralocorticoid replacement with fludrocortisone 5, 6.
• Patients with adrenal insufficiency are at risk of adrenal crisis, usually precipitated by major stress, and require careful management and education to avoid this life-threatening emergency 6.