Polyglandular autoimmune syndrome type 2: diagnosed in the intensive care unit

Abstract

Polyglandular autoimmune syndrome type 2 is a rare syndrome that commonly has the constellation of three diseases: diabetes mellitus type 1, hypothyroidism and adrenal insufficiency. Owing to the diabetes mellitus type 1, patients require life-long insulin therapy and blood glucose levels need to be monitored. They are at risk for chronic complications of diabetes such as neuropathy, nephropathy and retinopathy. More acutely, due to fluctuations in blood glucose levels, they are at risk for hypoglycemia with neuroglycopenic symptoms and ketoacidosis. Hypothyroidism is diagnosed by measuring the thyroid hormone levels and if inappropriately low is treated with replacement therapy. Patients with adrenal insufficiency experience symptoms due to low glucocorticoid and mineralocorticoid levels in the body, due to decreased or absent production. We present a case in which a patient presented with a picture of diabetic ketoacidosis was found to have hypothyroidism in her medical history and was diagnosed with adrenal insufficiency in the intensive care unit (ICU), later confirmed as an outpatient to be permanent. Based on the three diagnoses it was concluded that she has polyglandular autoimmune syndrome type 2.