Han Na Kim1, Betiel Fesseha1, Laura Anzaldi2, Allison Tsao3, Panagis Galiatsatos4, Aniket Sidhaye5. 1. Division of Endocrinology, Diabetes, & Metabolism, Department of MedicineThe Johns Hopkins University School of Medicine, Baltimore, Md. 2. The Johns Hopkins University School of Medicine, Baltimore, Md. 3. Department of MedicineThe Johns Hopkins University School of Medicine, Baltimore, Md. 4. Division of Pulmonary & Critical Care Medicine, The Johns Hopkins University School of Medicine, Baltimore, Md; Critical Care Department, National Institutes of Health, Bethesda, Md. 5. Division of Endocrinology, Diabetes, & Metabolism, Department of MedicineThe Johns Hopkins University School of Medicine, Baltimore, Md. Electronic address: asidhay1@jhmi.edu.
Abstract
BACKGROUND: Type 2 diabetes mellitus is characterized by relative insulin deficiency and insulin resistance. Features suggesting severe insulin resistance include acanthosis nigricans, hyperandrogenism, weight loss, and recurrent hospital admissions for diabetic ketoacidosis. In rare circumstances, hyperglycemia persists despite administration of massive doses of insulin. In these cases, it is important to consider autoimmune etiologies for insulin resistance, such as type B insulin resistance and insulin antibody-mediated extreme insulin resistance, which carry high morbidity and mortality if untreated. Encouragingly, immunomodulatory regimens have recently been published that induce remission at high rates. METHODS/ RESULTS: We describe 3 cases of extreme insulin resistance mediated by anti-insulin receptor autoantibodies or insulin autoantibodies. All cases were effectively treated with an immunomodulatory regimen. CONCLUSION: Although cases of extreme insulin resistance are rare, it is important to be aware of autoimmune causes, recognize suggestive signs and symptoms, and pursue appropriate diagnostic evaluation. Prompt treatment with immunomodulators is key to restoring euglycemia in patients with autoimmune etiologies of insulin resistance.
BACKGROUND: Type 2 diabetes mellitus is characterized by relative insulin deficiency and insulin resistance. Features suggesting severe insulin resistance include acanthosis nigricans, hyperandrogenism, weight loss, and recurrent hospital admissions for diabetic ketoacidosis. In rare circumstances, hyperglycemia persists despite administration of massive doses of insulin. In these cases, it is important to consider autoimmune etiologies for insulin resistance, such as type B insulin resistance and insulin antibody-mediated extreme insulin resistance, which carry high morbidity and mortality if untreated. Encouragingly, immunomodulatory regimens have recently been published that induce remission at high rates. METHODS/ RESULTS: We describe 3 cases of extreme insulin resistance mediated by anti-insulin receptor autoantibodies or insulin autoantibodies. All cases were effectively treated with an immunomodulatory regimen. CONCLUSION: Although cases of extreme insulin resistance are rare, it is important to be aware of autoimmune causes, recognize suggestive signs and symptoms, and pursue appropriate diagnostic evaluation. Prompt treatment with immunomodulators is key to restoring euglycemia in patients with autoimmune etiologies of insulin resistance.
Authors: Marcio José Concepción-Zavaleta; Sofía Pilar Ildefonso-Najarro; Esteban Alberto Plasencia-Dueñas; María Alejandra Quispe-Flores; Cristian David Armas-Flórez; Laura Esther Luna-Victorio Journal: Endocrinol Diabetes Metab Case Rep Date: 2020-10-14