Management of Adrenal Deficiency and Shock in a Patient With Polyglandular Autoimmune Syndrome Type II

Polyglandular autoimmune syndrome (PAS) is a rare disorder characterized by the autoimmune destruction of multiple endocrine glands. Type II PAS is the most common of the PAS subtypes and is characterized by Addison’s disease, autoimmune thyroid disease, and type I diabetes mellitus. Disease manifestations are predominantly seen in young adulthood with an emerging endocrine disorder; however, a host of other autoimmune conditions can also be present before endocrine organ dysfunction. Due to the complex nature of presentation and management, an important consideration in patient care involves a multidisciplinary team with the addition of an endocrinologist. A 21-year-old African American woman with a medical history of PAS-II presented during three hospitalizations with adrenal crisis, diabetic ketoacidosis (DKA), and myxedema. The common theme across admissions entails a spectrum of adrenal dysfunction, including shock, as well as glucose and thyroid abnormalities. During her first hospitalization, the patient presented with hypotension, hyperglycemia, and hypothyroidism. She received aggressive IV fluid resuscitation, an insulin drip, electrolyte repletion, an up-titration of levothyroxine, and stress-dose corticosteroids. In the second hospitalization, she also had hypotension and electrolyte derangements, along with hypoglycemia and myxedema. She received glucose management, thyroid hormone replacement, and stress steroids again. The third hospitalization involved flu-like symptoms and a positive SARS-CoV-2 test. She was managed similarly for hypotension, hyponatremia, and hyperglycemia. In this case, she presented with non-gap metabolic acidosis and required a bicarbonate drip for a short period. She did not receive antibiotics across these three admissions. We present three hospitalizations where adrenal, pancreatic, and thyroid derangements were seen and managed. It is known that most general providers other than endocrinologists are not comfortable with the management of disease manifestations of PAS-II; therefore, we provide a case review to address the standard of care management and guidelines with further discussion. This patient’s maintenance care was complicated by a lack of adherence to outpatient medications, leading to recurrent hospitalizations. We also endorse the importance of doctors pursuing endocrinology fellowships, especially due to the observed waning number of graduates. An endocrinologist’s availability and involvement in the care of patients with complex endocrine issues lead to improved outcomes.