What happens if a patient on long-term steroids (corticosteroids) suddenly stops taking them and what labs, such as cortisol levels, electrolyte panels, and blood glucose, should be checked, and to which specialty, specifically an endocrinologist, should the patient be referred for proper treatment?

Management of Adrenal Insufficiency Following Abrupt Steroid Discontinuation

Patients who abruptly discontinue long-term steroid therapy should be immediately evaluated for adrenal insufficiency and referred to an endocrinologist for proper management, as sudden steroid withdrawal can lead to life-threatening adrenal crisis. 1

Clinical Manifestations of Adrenal Insufficiency

Abrupt discontinuation of long-term systemic steroids leads to adrenal insufficiency due to hypothalamic-pituitary-adrenal (HPA) axis suppression. Early recognition of symptoms is critical as volume-resistant hypotension may be a late or even agonal event 2.

Signs and symptoms include:

• Non-specific malaise
• Somnolence or altered mental status
• Cognitive dysfunction
• Orthostatic hypotension progressing to supine hypotension and shock
• Hyponatremia
• Hyperkalemia
• Hypoglycemia
• Persistent unexplained fever
• Nausea and vomiting
• Weakness and fatigue

Laboratory Evaluation

The following tests should be performed immediately:

• Electrolytes (sodium, potassium, chloride, bicarbonate)
• Blood glucose
• Morning cortisol level (though this may not be diagnostic in patients on corticosteroids)
• ACTH level (to differentiate between primary and secondary adrenal insufficiency)
• Cortisol stimulation test (if patient is stable enough) 1

Immediate Management

For patients with severe symptoms or suspected adrenal crisis:

1. Administer IV hydrocortisone 100 mg every 6-8 hours immediately
2. Provide IV normal saline for volume depletion and hyponatremia correction
3. Monitor vital signs, including orthostatic blood pressure measurements
4. Check electrolytes frequently 1

For patients with less severe symptoms:

1. Initiate oral hydrocortisone replacement at 15-20 mg daily in divided doses (10-15 mg in morning, 5 mg in early afternoon)
2. Add fludrocortisone 0.05-0.1 mg once daily if primary adrenal insufficiency is suspected 1

Long-term Management and Referral

All patients with adrenal insufficiency following steroid discontinuation should be referred to an endocrinologist for:

• Adjustment of hydrocortisone dose based on clinical response (maximum daily dose of 30 mg)
• Education on stress-dose protocols (doubling or tripling hydrocortisone dose during illness)
• Prescription of emergency injectable hydrocortisone
• Recommendation for medical alert bracelet/necklace
• Testing for HPA axis recovery after 3 months of maintenance therapy 1

Prevention of Adrenal Insufficiency

To prevent adrenal insufficiency when discontinuing steroids:

• Taper steroids gradually, reducing by no more than 2.5-5 mg every 1-2 weeks
• For patients who have been on prolonged therapy, taper even more slowly
• Stress-dose steroids should be tapered over 5-7 days to maintenance doses 1, 3

Special Considerations

• Patients with adrenal insufficiency are at risk during periods of physiological stress and should follow "Sick Day Rules" by doubling steroid doses during illness 2
• Patients should be educated about the symptoms of adrenal insufficiency and when to seek medical attention
• The risk of adrenal insufficiency varies according to dose from 2.4% (low dose) to 21.5% (high dose), and according to treatment duration from 1.4% (<28 days) to 27.4% (>1 year) 4
• Recovery of adrenal function typically occurs within 6-12 months but can take up to 51 months in some cases 5, 6
• Approximately 5% of patients may never recover normal adrenal function 6

Pitfalls to Avoid

• Do not delay treatment in patients with suspected adrenal crisis while waiting for confirmatory testing
• Do not start thyroid hormone replacement before cortisol replacement in patients with multiple hormone deficiencies
• Do not assume patients are fully educated about sick day rules or adrenal crisis management
• Be vigilant for hypercalcemia, which can be a rare presentation of glucocorticoid-induced adrenal insufficiency 7
• Never abruptly discontinue steroids in patients who have been on long-term therapy 3

By following these guidelines, clinicians can effectively manage patients who have developed adrenal insufficiency following abrupt steroid discontinuation and prevent potentially life-threatening complications.