Endocrine Abstracts

We present the case of a 58-year-old Caucasian woman with a medical history of Hashimoto’s hypothyroidism who presented with several months of orthostasis, unintentional weight loss, nausea, and vomiting. Initial laboratory investigations revealed severe hyponatremia to 106 mmol/l, which prompted admission to the intensive care unit. TSH and free T4 were normal. She had orthostatic hypotension. Her outpatient medications were levothyroxine 50 mg daily and as-needed ondansetron which her integrative wellness doctor recently prescribed for nausea and vomiting. On examination, she was noted to be underweight with generalized skin hyperpigmentation, including over non-sun-exposed areas of the body such as the back, buttocks, and palmar creases. Investigations revealed autoimmune primary adrenal insufficiency as the cause of her symptoms. She was initiated on treatment with glucocorticoid and mineralocorticoid replacement therapy, with normalization of her sodium levels. Latent autoimmune diabetes mellitus was also uncovered due to significant hyperglycemia while she was receiving stress dose steroids. Assays were markedly positive for 21-hydroxylase, thyroid peroxidase, and glutamic acid decarboxylase antibodies. Due to the combination of autoimmune primary adrenal insufficiency, Hashimoto’s hypothyroidism, and latent autoimmune diabetes mellitus, she was given a diagnosis of autoimmune polyglandular syndrome type 2 (APS 2). APS 2 is a rare autoimmune-mediated disease that affects a cluster of different endocrine glands. It is typically characterized by the primary presence of primary adrenocortical insufficiency associated with autoimmune thyroid disease and/or autoimmune type 1 diabetes mellitus (T1DM). Other autoimmune conditions may also be involved. It is a rare disease that may occur at any age but most commonly between 30-40 years, and women tend to be more affected than men. In about half the cases, adrenocortical insufficiency is the initial endocrine abnormality. Our patient was counseled extensively on this diagnosis and was discharged home with instructions for outpatient endocrinology follow-up.