A Rare Case of Autoimmune Polyglandular Syndrome Type 2 in a Child With Persistent Fatigue

Smith, Ryan Kenneth; Gerrits, Peter M.

Further history revealed recent gait instability as well as a change in skin color for a few weeks preceding presentation. Physical examination was significant for generalized pallor and a dusky gray appearance, generalized abdominal tenderness, and right-sided CVA tenderness. Because hyponatremia in the context of nausea, vomiting, fatigue, and skin color changes raised suspicion for Addison's disease, a comprehensive metabolic panel and early morning cortisol were ordered. The next day he had documented pigmentation of the lower lip and both nipples. Further laboratory evaluation revealed positive thyroid peroxidase antibody, elevated ACTH, cortisol levels nonresponsive to cosyntropin, and persistent hyponatremia and hyperkalemia (see the Appendix for pertinent laboratory values.). Laboratory data were consistent with primary adrenal insufficiency and Hashimoto's thyroiditis. Hydrocortisone and fludrocortisone were initiated for glucocorticoid and mineralocorticoid replacement, respectively. Because adrenal dysfunction and acute illness can elevate the TSH, the putative Hashimoto's disease was not treated. Thyroid hormone replacement was also held to prevent adrenal crisis from stimulation of the patient's metabolism with thyroid hormone. A respiratory virus panel was positive for coronavirus, which might have precipitated the acute illness. Direct renin level was elevated, and the aldosterone level was low. By day 3, his electrolyte abnormalities and clinical status improved significantly, so he was discharged.