MON-876 - Autoimmune Polyendocrine Syndrome Type 3 through the Lens of Snow White and the Seven Dwarfs

Introduction:
Autoimmune polyendocrine syndrome (APS) represents immune mediated disorders characterized by sequential involvement of multiple endocrine and non-endocrine organs. We present the case of a 49-year-old woman with APS type 3 (APS-3), whose disease course illustrates the progressive, multisystem nature of autoimmunity and the importance of anticipatory surveillance. To aid clinical recall, APS is compared to snow white and the seven dwarfs, where each dwarf represents a potential endocrine target of autoimmune attack.

Case presentation:
We present a case of a 49-year-old women who has established care with endocrinology for her history of APS-3 which was characterized by type 1 diabetes mellitus (DM), Hashimoto thyroiditis, pernicious anemia secondary to vitamin B12 deficiency, and myasthenia gravis. Her course began with myasthenia gravis with previous episodes of myasthenic crisis which has required complex immunomodulatory therapy by neurology. She then subsequently developed type 1 DM with symptoms of polyuria and polyphagia complicated with diabetic ketoacidosis (glucose 659 mg/dL) with electrolyte abnormalities. She is now managed on a tandem Mobi insulin pump with Dexcom G7 CGM. Glycemic control has been generally acceptable but complicated by intermittent steroid exposure and patient-initiated pump overrides, resulting in episodes of hypoglycemia. Recent CGM data demonstrate a time-in-range of 74% with a GMI of approximately 7.1%.
Her Hashimoto thyroiditis is well controlled on levothyroxine 112 mcg, with TSH at goal 2.702 uIU/mL (0.550 – 4.780). Thyroid autoimmunity was previously confirmed by markedly elevated thyroglobulin antibody titers 52 IU/mL (normal < 20 IU/mL). She had no clinical or biochemical evidence of adrenal insufficiency; 21-hydroxylase antibodies are negative, and prior adrenal testing was reportedly normal.
She also carries diagnosis of pernicious anemia with vitamin B12 deficiency 149 pg/mL (193-986), managed with intramuscular B12 injections. In addition, she has premature ovarian insufficiency, with menopause occurring at age 38–39, now treated with estrogen and progesterone replacement therapy through gynecology.

Discussion:
Overall, this patient demonstrates a classic and evolving presentation of APS-3, with significant multisystem involvement requiring close multidisciplinary coordination. In our analogy, snow white represents the immune system who gets misguided and attacks her own kingdom. Each dwarf represents an endocrine or autoimmune target (Doc – Pituitary, Grumpy – Adrenal, Sleepy – Thyroid, Happy-Pancreas, Bashful-Gonads, Sneezy-Parathyroids, and Dopey-other autoimmune conditions). Just as snow white is not defined by one dwarf alone, the diagnosis of one autoimmune endocrine disease should prompt systematic screening and longitudinal surveillance of others-because another dwarf may be next.

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