Insulin Receptor Autoantibody-mediated Hypoglycemia in a Woman With Mixed Connective Tissue Disease.
Max C PetersenJonah M GravesTony YaoLutz SchomburgWaldemar B MinichDeborah L ParksJanet B McGillMaamoun SalamPublished in: Journal of the Endocrine Society (2021)
Autoantibodies to the insulin receptor are rare and typically cause severe insulin resistance and hyperglycemia, a condition termed type B insulin resistance . Uncommonly, antibodies to the insulin receptor can cause hypoglycemia. We present the case of a woman who developed recurrent severe hypoglycemia and myopathy, was found to have insulin receptor autoantibodies and mixed connective tissue disease, and had resolution of hypoglycemia with immunosuppression. A 55-year-old woman with a history of obesity, hypertension, and prior hemorrhagic stroke presented with recurrent severe hypoglycemia. A diagnostic fast resulted in hypoinsulinemic hypoketotic hypoglycemia. Adrenal function was intact. Progressive myopathy had developed simultaneously with her hypoglycemia, and rheumatologic evaluation revealed mixed connective tissue disease. The plasma acylcarnitine profile was normal, extensive oncologic evaluation including insulin-like growth factor 2 measurement was unrevealing, and anti-insulin antibody testing was negative. Ultimately, anti-insulin receptor antibodies were found to be present. The patient was treated with glucocorticoids and rituximab. Eight weeks after initiation of immunosuppression, the insulin receptor antibody titer had decreased and hypoglycemia had resolved. Eight months after diagnosis, the patient remained free of severe hypoglycemia despite tapering of glucocorticoids to a near-physiologic dose. Though antibodies to the insulin receptor typically cause severe insulin resistance, this patient had no evidence of insulin resistance and instead presented with recurrent severe hypoglycemia, which responded to glucocorticoids and rituximab. The diagnosis of insulin receptor antibody-mediated hypoglycemia is rare but should be considered in patients with systemic autoimmune disease, including mixed connective tissue disease, in the appropriate clinical context.
Keyphrases
- type diabetes
- glycemic control
- insulin resistance
- early onset
- metabolic syndrome
- high fat diet
- multiple sclerosis
- adipose tissue
- weight loss
- polycystic ovary syndrome
- prostate cancer
- systemic lupus erythematosus
- body mass index
- drug induced
- skeletal muscle
- diffuse large b cell lymphoma
- blood pressure
- single molecule
- late onset
- blood brain barrier
- preterm birth