A case of Nelson's syndrome with adrenal crisis occurring 21 years post-adrenalectomy for Cushing's disease.

BACKGROUND: Nelson’s syndrome is a known complication of bilateral adrenalectomy for Cushing’s disease, and lifelong glucocorticoid replacement is essential in these patients to prevent adrenal insufficiency, a condition that can culminate in life-threatening adrenal crisis. This medical emergency, often precipitated by stressors like infection, can be diagnostically challenging as it mimics septic shock. The co-occurrence of Nelson’s syndrome and adrenal crisis is exceptionally rare. To our knowledge, we report the first case of a patient with Nelson’s syndrome who developed an adrenal crisis 21 years post-adrenalectomy, highlighting a critical long-term risk. CASE PRESENTATION: This case describes a 44-year-old woman with a one-week history of progressive anorexia, nausea, vomiting, and fatigue, followed by three days of fever (peak 38.8℃) and altered mental status.Despite active anti-infection and fluid replacement support treatments, shock could not be corrected and a diagnosis of adrenal crisis was identified only after detailed medical history tracing and complete determination of hormone levels.The improvement of refractory shock by glucocorticoid therapy, emphasizes that the possibility of adrenal crisis should be considered when shock cannot be corrected routinely. Lifelong monitoring of Nelson patients plays an extremely important role in preventing the occurrence of tragedies. CONCLUSION: This case highlights adrenal crisis should be taken into account when shock cannot be routinely corrected. A detailed patient’s history may be crucial for a correct diagnosis and treatment and lifelong monitoring is necessary for patients with adrenal crisis. CLINICAL TRIAL NUMBER: Not applicable.