Delayed diagnosis with autoimmune polyglandular syndrome type 2 causing acute adrenal crisis: A case report

Abstract

Background: Autoimmune polyglandular syndrome type 2 (APS-2), also known as Schmidt's syndrome, is an uncommon disorder characterized by the coexistence of Addison's disease with thyroid autoimmune disease and/or type 1 diabetes mellitus. Addison's disease as the obligatory component is potentially life-threatening. Unfortunately, the delayed diagnosis of Addison's disease is common owing to its rarity and the nonspecific clinical manifestation.

Methods: Here we reported a case of 38-year-old female patient who presented with 2 years' history of Hashimoto's thyroiditis and received levothyroxine replacement. One year later, skin hyperpigmentation, fatigue, loss of appetite, and muscle soreness occurred. She was advised to increase the dose of levothyroxine, but the symptoms were not relieved. After 4 months, the patient accompanied with dizziness, nausea, nonbloody vomiting, and fever. However, she was diagnosed with acute gastroenteritis and fell into shock and ventricular fibrillation subsequently. Further evaluation in our hospital revealed elevated adrenocorticotrophic hormone and low morning serum cortisol, associated with hyponatremia and atrophic adrenal gland. Hypergonadotropic hypogonadism and Hashimoto's thyroiditis were also demonstrated.

Results: After the supplementation with hydrocortisone and fludrocortisone was initiated, the physical discomforts were alleviated and plasma electrolytes were back to normal.

Conclusion: The uncommon case involving 3 endocrine organs reinforced the significance of a timely diagnosis and appropriate treatment of APS-2, and physicians needed to sharpen their awareness of the potentially life-threatening disease.

Figure 1

Computed tomography imaging of the adrenal glands. The arrows showed the remaining atrophic adrenal glands.