SFEBES2016 Oral Communications Diabetes Mellitus and Metabolism (6 abstracts)
Two contrasting cases of spontaneous severe hypoglycaemia secondary to anti-insulin antibodies (Insulin Autoimmune Syndrome / Hirata disease)
Myuri Moorthy 1 , Bernard Freudenthal 3 , David Church 2 , Judith Kisalu 1 , Emma Woolman 1 , Amy Hale 1 , Huw Beynon 1 , Efthimia Karra 1 , Mark Cohen 3 & Bernard Khoo 1
1Department of Endocrinology & Diabetes, Royal Free Hospital London NHS Foundation Trust, London, UK; 2Department of Clinical Biochemistry, University of Cambridge, Cambridge, UK; 3Department of Endocrinology and Diabetes, Barnett Hospital, London, UK.
IAS is a very rare condition in which anti-insulin antibodies carry high concentrations of insulin in suspension in circulation. Hypoglycaemia occurs when insulin is released from the antibodies during fasting or post-prandially.
We present two cases. Patient-A is a 52-year old Thai obese female, with acanthosis nigricans and a strong family history of T2DM. Patient-B is a 28 year-old normal- BMI Caucasian female with an unremarkable history. None of the patients received any regular medication or had history of autoimmunity. Both presented with symptomatic hypoglycaemia. Whipple’s triad was noted at 10 hr of a supervised fast for patient-A. Nadir laboratory glucose was 1.8 mmol/l, and coupled with hyperinsulinaemia and a non-physiological ratio of insulin-to- C-peptide (insulin=9809 mIU, C-peptide=3690 pmol/l, insulin:C-peptide=18.5). Patient-B developed hypoglycaemia at 4 hr during a supervised fast, with hyperinsulinaemia and a high ratio of insulin-to- C-peptide (plasma glucose=2.2 mmol/l, insulin=17800 pmol/l, C-peptide=409 pmol/l, insulin:C-peptide- ratio=43.5 [normal insulin:C-peptide ratio ≤1]). Hook-effect phenomena were excluded with insulin/C-peptide recovery post-serial dilutions. Insulin was lower post-PEG precipitation. SU screen was negative and CT CAP, MRI & Ga68-DOTATATE unremarkable. Anti-insulin-receptor Abs were negative, whereas anti-insulin IgG were positive. Chromatography demonstrated insulin-sequestration by Ab, identifying monomeric and Ab-bound insulin. Pending diagnosis, both patients received diazoxide with no efficacy. In light of positive insulin Ab, prednisolone 30 mg and add-on mycophenolate (MMF) treatment were initiated in patient-A; with later euglycaemia maintained on MMF monotherapy. In patient-B prednisolone 60 mg and add-on MMF only induced partial response, thus, CD20 depletion by Rituximab steroid adjuvant treatment strategy was adopted with efficacy.
We discuss the diagnostic challenges in IAS, the diverse phenotype and treatment responses in our two cases.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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