Polyglandular Syndrome with Complications and a Rare Co-existence of Hypercortisolism in a Young Girl: An Internist Approach

Abstract

Patients diagnosed with Type 1 Diabetes Mellitus (T1DM) can sometimes manifest as part of broader clinical presentations known as Autoimmune Polyglandular Syndromes (APS). APS refers to a group of rare autoimmune disorders in which multiple endocrine glands are affected by autoimmune attacks. There are four types of APS described so far, including APS1, APS2, APS3 and APS4. In contrast to APS1 and APS2, APS3 does not involve the adrenal cortex. In APS3, autoimmune thyroiditis occurs with other organspecific autoimmune diseases, excluding Addison’s disease. A 20-year-old female with a known case of T1DM, Chronic Kidney Disease (CKD), hypothyroidism, and hypertension recently presented with a hypertensive emergency. On further evaluation, she was also diagnosed as a possible case of APS3 with a rare presentation of hypercortisolism instead of Addison’s disease. Various challenges were faced while managing her diabetes due to the brittle diabetes pattern she showed, and also there was a dilemma in the conclusive diagnosis of hypercortisolism in this patient due to the co-existing CKD.

Keywords

• chronic kidney disease
• hypothyroidism
• hypercortisolism
• hypertensive emergency
• type 1 diabetes mellitus