An unusual presentation of primary adrenal insufficiency with new onset type 1 diabetes: case report and review of the literature

Abstract

Objectives: To describe an atypical presentation of primary adrenal insufficiency in conjunction with new onset type 1 diabetes.

Case presentation: Here, we describe a case of new-onset type 1 diabetes (T1D) presenting simultaneously with an unusual presentation of primary adrenal insufficiency in a previously healthy 16-year-old. He was admitted for a typical presentation of diabetic ketoacidosis, but with extreme hyponatremia. An extensive workup revealed a low aldosterone level, appropriate cortisol level, and positive 21-hydroxylase antibodies. While the phenomenon of multiple autoimmune conditions developing in the same patient is well-described, this particular case has several atypical aspects. Our patient's case highlights the danger of relying on random serum cortisol in the setting of acute illness to rule out adrenal insufficiency.

Conclusions: Adrenal insufficiency can present as isolated hypoaldosteronism without hypocortisolemia and can manifest as severe hyponatremia in the context of diabetic ketoacidosis. Workup for an unusual presentation of T1D should include a 21-hydroxylase antibody, as well as thyroid and celiac disease studies.

Keywords: Addison’s disease; autoimmune polyglandular syndrome; primary adrenal insufficiency; type 1 diabetes mellitus.