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2016 ; 95
(42
): e5062
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Delayed diagnosis with autoimmune polyglandular syndrome type 2 causing acute
adrenal crisis: A case report
#MMPMID27759634
Wang X
; Ping F
; Qi C
; Xiao X
Medicine (Baltimore)
2016[Oct]; 95
(42
): e5062
PMID27759634
show ga
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.