Diabetes Metab J.  2011 Feb;35(1):80-85. 10.4093/dmj.2011.35.1.80.

Autoimmune Hypoglycemia in a Patient with Characterization of Insulin Receptor Autoantibodies

• Chon S
• Choi MC
• Lee YJ
• Hwang YC
• Jeong IK
• Oh S
• Ahn KJ
• Chung HY
• Woo JT
• Kim SW
• Kim JW
• Kim YS

• KMID: 2281496
• DOI: http://doi.org/10.4093/dmj.2011.35.1.80

Abstract

BACKGROUND
Type B insulin resistance syndrome is a manifestation of autoantibodies to the insulin receptor that results in severe hyperglycemia and acanthosis nigricans. However, the mechanisms by which these autoantibodies induce hypoglycemia are largely unknown. In this paper, we report the case of patient with type B insulin resistance syndrome who presented with frequent severe fasting hypoglycemia and acanthosis nigricans.
METHODS
To evaluate the mechanism of hypoglycemia, we measured the inhibition of insulin binding to erythrocytes and IM9 lymphocytes in a sample of the patient's dialyzed serum before and after immunosuppressive therapy.
RESULTS
In the patient's pre-treatment serum IgG, the binding of 125I-insulin to erythrocytes was markedly inhibited in a dose-dependent manner until the cold insulin level reached 10-9 mol/L. We also observed dose-dependent inhibition of insulin binding to IM9 lymphocytes, which reached approximately 82% inhibition and persisted even when diluted 1:20. After treatment with glucocorticoids, insulin-erythrocyte binding activity returned to between 70% and 80% of normal, while the inhibition of insulin-lymphocyte binding was reduced by 17%.
CONCLUSION
We treated a patient with type B insulin resistance syndrome showing recurrent fasting hypoglycemia with steroids and azathioprine. We characterized the patient's insulin receptor antibodies by measuring the inhibition of insulin binding.

Keyword

Autoimmune hypoglycemia; Insulin receptor antibody; Type B insulin resistance

MeSH Terms

Acanthosis Nigricans
Antibodies
Autoantibodies
Azathioprine
Cold Temperature
Erythrocytes
Glucocorticoids
Humans
Hyperglycemia
Hypoglycemia
Immunoglobulin G
Insulin
Insulin Resistance
Lymphocytes
Receptor, Insulin
Steroids
Antibodies
Autoantibodies
Azathioprine
Glucocorticoids
Immunoglobulin G
Insulin
Receptor, Insulin
Steroids

Figure

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  Fig. 1 Thickened, hyperpigmented skin lesions (acanthosis nigricans) were observed on the posterior neck, axillae and groin.

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  Fig. 2 Initial 24-hour glucose profile using a continuous glucose monitoring system during a 72-hour fasting test.

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  Fig. 3 Pre- and post-treatment serum levels of insulin and C-peptide after 75 g oral glucose tolerance test. (A) On admission. (B) Sixteen-month follow-up.

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  Fig. 4 Post-treatment 24-hour glucose profile using a continuous glucose monitoring system. (A) After plasmapheresis. (B) After steroid treatment.

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  Fig. 5 Effect of serum IgG on insulin binding of erythrocytes.

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  Fig. 6 Effect of serum IgG on insulin binding of IM9 lymphocytes.

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