A Case of Type B Insulin Resistance Syndrome Accompanied with Mixed Connective Tissue Disease and Hashimoto's Thyroiditis

2008 ◽  
Vol 9 (3) ◽  
pp. 228
Author(s):  
Sang Ryul Lee ◽  
Bo Kyung Koo ◽  
Eun kyoung Park ◽  
Eun Ju Song ◽  
Dae Hyun Baek ◽  
...  
Keyword(s):  
Insulin Resistance ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Hashimoto’S Thyroiditis ◽  
Mixed Connective Tissue Disease ◽  
Insulin Resistance Syndrome ◽  
Hashimoto's Thyroiditis ◽  
Type B

A case of mixed connective tissue disease complicated with thymic carcinoma and Hashimoto's thyroiditis

2007 ◽  
Vol 17 (1) ◽  
pp. 63-66 ◽  
Author(s):  
Yoshito Yoshidome ◽  
Shigeaki Hayashi ◽  
Yoshikazu Maruyama ◽  
Toshi Yoneda ◽  
Daisuke Matsuzoe ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Hashimoto’S Thyroiditis ◽  
Mixed Connective Tissue Disease ◽  
Thymic Carcinoma ◽  
Hashimoto's Thyroiditis

Type B insulin resistance syndrome in a patient with connective tissue disease

2017 ◽  
Author(s):  
Agnieszka Lebkowska ◽  
Anna Krentowska ◽  
Agnieszka Adamska ◽  
Beata Piasecka ◽  
Danuta Lipinska ◽  
...  
Keyword(s):  
Insulin Resistance ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Insulin Resistance Syndrome ◽  
Type B

scholarly journals Type B insulin resistance syndrome associated with connective tissue disease and psoriasis

2020 ◽  
Vol 2020 ◽  
Author(s):  
Agnieszka Łebkowska ◽  
Anna Krentowska ◽  
Agnieszka Adamska ◽  
Danuta Lipińska ◽  
Beata Piasecka ◽  
...  
Keyword(s):  
Insulin Resistance ◽  
Connective Tissue ◽  
Insulin Receptor ◽  
Connective Tissue Disease ◽  
Acanthosis Nigricans ◽  
Insulin Resistance Syndrome ◽  
List Type ◽  
Type B ◽  
Immunoprecipitation Assay ◽  
Severe Insulin Resistance

Summary Type B insulin resistance syndrome (TBIR) is characterised by the rapid onset of severe insulin resistance due to circulating anti-insulin receptor antibodies (AIRAs). Widespread acanthosis nigricans is normally seen, and co-occurrence with other autoimmune diseases is common. We report a 27-year-old Caucasian man with psoriasis and connective tissue disease who presented with unexplained rapid weight loss, severe acanthosis nigricans, and hyperglycaemia punctuated by fasting hypoglycaemia. Severe insulin resistance was confirmed by hyperinsulinaemic euglycaemic clamping, and immunoprecipitation assay demonstrated AIRAs, confirming TBIR. Treatment with corticosteroids, metformin and hydroxychloroquine allowed withdrawal of insulin therapy, with stabilisation of glycaemia and diminished signs of insulin resistance; however, morning fasting hypoglycaemic episodes persisted. Over three years of follow-up, metabolic control remained satisfactory on a regimen of metformin, hydroxychloroquine and methotrexate; however, psoriatic arthritis developed. This case illustrates TBIR as a rare but severe form of acquired insulin resistance and describes an effective multidisciplinary approach to treatment. Learning points: We describe an unusual case of type B insulin resistance syndrome (TBIR) in association with mixed connective tissue disease and psoriasis. Clinical evidence of severe insulin resistance was corroborated by euglycaemic hyperinsulinaemic clamp, and anti-insulin receptor autoantibodies were confirmed by immunoprecipitation assay. Treatment with metformin, hydroxychloroquine and methotrexate ameliorated extreme insulin resistance.

scholarly journals Insulin receptor autoantibody-mediated hypoglycemia in a woman with mixed connective tissue disease

2021 ◽  
Author(s):  
Max C Petersen ◽  
Jonah M Graves ◽  
Tony Yao ◽  
Lutz Schomburg ◽  
Waldemar B Minich ◽  
...  
Keyword(s):  
Insulin Resistance ◽  
Connective Tissue ◽  
Insulin Receptor ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Severe Hypoglycemia ◽  
Systemic Autoimmune Disease ◽  
Antibody Testing ◽  
Severe Insulin Resistance ◽  
Receptor Antibody

Abstract 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 IGF-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.

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