From the Guidelines
Management of a patient with low plasma ACTH levels should focus on treating secondary adrenal insufficiency with glucocorticoid replacement therapy, typically with oral hydrocortisone at a total daily dose of 15-25 mg divided into 2-3 doses. This approach is based on the most recent and highest quality study available, which provides guidance on the management of immune-related adverse events in patients treated with immune checkpoint inhibitor therapy 1. The study recommends initiating replacement therapy with hydrocortisone (15-20 mg in divided doses) and titrating to a maximum of 30 mg daily total dose for residual symptoms of adrenal insufficiency.
Key considerations in the management of low plasma ACTH levels include:
- Educating patients on stress dosing for sick days, use of emergency injectables, and when to seek medical attention for impending adrenal crisis 1
- Providing a medical alert bracelet or necklace for adrenal insufficiency to trigger stress-dose corticosteroids by emergency medical personnel 1
- Endocrine consultation prior to surgery or any procedure for stress-dose planning 1
- Regular monitoring for signs of over-replacement (weight gain, hypertension, glucose intolerance) or under-replacement (fatigue, nausea, hypotension) 1
The underlying cause of low ACTH should be investigated, as it may be due to exogenous glucocorticoid use, pituitary tumors, or other hypothalamic-pituitary disorders that might require additional specific treatments 1. Mineralocorticoid replacement is usually unnecessary in secondary adrenal insufficiency, as aldosterone production remains intact 1.
From the Research
Management Approach for Low Plasma ACTH Level
The management approach for a patient with a low plasma Adrenocorticotropic Hormone (ACTH) level involves several key considerations:
- Diagnosis: The diagnosis of isolated ACTH deficiency is made by demonstrating low cortisol production with low plasma ACTH, absent adrenal responses to stimulation for pituitary or hypothalamus with intact adrenal response to exogenous ACTH, and normal secretory indices of other pituitary hormones 2.
- Treatment: Treatment for secondary adrenal insufficiency in ACTH isolated deficiency involves administering hydrocortisone, which can lead to recovery of cardiac dysfunction in cases of ampulla (takotsubo) cardiomyopathy 3.
- Monitoring and Adjustment: In patients with known adrenal insufficiency, it is crucial to adjust the glucocorticoid dosage during intercurrent illness to prevent life-threatening situations, and patients should be instructed on how to deal with physical and severe psychic stress 4.
- Chronic Replacement Therapy: Chronic replacement therapy with glucocorticoids and, in primary adrenal insufficiency, mineralocorticoids requires careful monitoring, and current replacement strategies still require optimization 5.
- Recognition and Prompt Treatment: Central adrenal insufficiency (CAI) recognition and prompt treatment are vital, and the diagnosis of CAI is challenging due to its oligosymptomatic clinical presentation and laboratory investigation pitfalls 6.
Key Considerations
- Patients with low plasma ACTH levels require careful monitoring and adjustment of their treatment plans to prevent life-threatening complications.
- The diagnosis of isolated ACTH deficiency and central adrenal insufficiency can be challenging, and a stepwise diagnostic approach is necessary.
- Treatment with hydrocortisone and other glucocorticoids can be effective in managing secondary adrenal insufficiency and CAI.
- Patient education and instruction on how to deal with physical and severe psychic stress are crucial in preventing unnecessary deaths from Addisonian crisis.