Insulin Autoimmune Syndrome (Hirata Disease)

Uchigata, Yasuko; Hirata, Yoshihiro

doi:10.1007/978-1-60327-478-4_21

Cited by 76 publications

(139 citation statements)

References 52 publications

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“…About half of IAS patients report recent exposure to medications, with over 90% of offending agents containing a thiol group such as Methimazole (anti-thyroid agent), Penicillamine, Glutathione, Hydralazine, Procainamide and Isoniazid [4,8]. The subject described in the present report was taking two drugs that contained active thiol metabolites, namely Pantoprazole and Clopidogrel.…”

Section: Case Reportmentioning

confidence: 79%

“…The cases reported in Japan have shown strong association between IAS and HLA-DR4 status, haplotype 20 times more common in Asians compared with non-Asian subjects, or the use of medications containing sulfhydryl (thiol) group. Cases reported from other parts of the world are more frequently associated with autoimmune disorders and plasma cell dyscrasias [3,4]. The majority of the cases reported outside Asia come from Europe (50%) and the United States (41%).…”

Section: Introductionmentioning

confidence: 99%

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A Case of Autoimmune Hypoglycemia in Switzerland

Stefanescu¹,

Bart²

2018

J Clin Case Rep

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In this report, we describe a white man with symptomatic hypoglycemia whose medical work-up revealed an excessively elevated serum insulin level (867 mIU/l). The need to measure IAA in patients with extraordinarily high measured levels of total insulin is essential because of the possible artifactual elevation (in a conventional insulin immunoassay) caused by IAA. In the present case, the elevated IAA level (>50 kIU/l) confirmed the diagnosis of autoimmune hypoglycemia and allowed to avoid unnecessary surgical intervention.

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Section: Case Reportmentioning

confidence: 79%

Section: Introductionmentioning

confidence: 99%

A Case of Autoimmune Hypoglycemia in Switzerland

Stefanescu¹,

Bart²

2018

J Clin Case Rep

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“…2 First described by Hirata et al in 1970, 3 it is the third leading cause of spontaneous hypoglycemia in Japan following insulinoma and extrapancreatic neoplasms. 4 Over 380 cases have been reported in the medical literature since, 5 with the majority (90 %) depicted in the Japanese population.…”

Section: Discussionmentioning

confidence: 99%

Recurrent Hypoglycemia from Insulin Autoimmune Syndrome

2013

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Insulin autoimmune syndrome (IAS) is an uncommon cause of hyperinsulinemic hypoglycemia characterized by autoantibodies to endogenous insulin in individuals without previous exposure to exogenous insulin. IAS is the third leading cause of spontaneous hypoglycemia in Japan, and is increasingly being recognized worldwide in non-Asian populations. We report a case of IAS in a Caucasian woman with recurrent complaints of hypoglycemia, with laboratory findings of serum glucose 2.5 mmol/L (45 mg/dL), insulin 54,930 pmol/L (7,909 μIU/mL), connecting peptide (C-peptide) 4,104 pmol/L (12.4 ng/mL), and a corresponding insulin to C-peptide molar ratio of 13.4 during a spontaneous hypoglycemic event. Autoantibodies to insulin were markedly elevated at > 50 kU/L (> 50 U/mL). IAS should be considered in the differential diagnosis of hypoglycemia in non-diabetic individuals. Distinction from insulinoma is especially crucial to prevent unwarranted invasive procedures and surgical interventions in hypoglycemic patients.KEY WORDS: insulin autoimmune syndrome; hypoglycemia; insulin to C-peptide molar ratio.

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“…However, in the event of negative localisation studies (including advanced molecular imaging techniques) it is important to consider other differential diagnoses that may biochemically masquerade as endogenous hyperinsulinaemic hypoglycaemia (Paiva et al 2006). Insulin autoimmune syndrome (IAS) (Uchigata & Hirata 1999) is most commonly identified in Asian populations (particularly Japan) and is usually associated with post-prandial hypoglycaemia. It is characterised by markedly elevated insulin levels and may be associated with other autoimmune diseases.…”

Section: Clinical Hypoglycaemia Syndromesmentioning

confidence: 99%

Molecular imaging in the investigation of hypoglycaemic syndromes and their management

Pattison¹,

Hicks²

2017

Endocrine-Related Cancer

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There has been recent progress in molecular imaging using a variety of cellular targets for the investigation of adult non-diabetic hypoglycaemic syndromes and its integration into patient management. These targets include peptide receptors (somatostatin receptors (SSTRs) and glucagon-like peptide-1 receptor (GLP-1R)) the amine precursor uptake and decarboxylation system utilising the diphydroxyphenylaline (DOPA) analogue 6-[ 18 F]-l-fluoro-l-3,4-dihydroxyphenylalanine ( 18 F-FDOPA), and glycolytic metabolism with 2-[ 18 F]fluoro-2-deoxy-d-glucose (FDG). Accurate preoperative localisation and staging is critical to enable directed surgical excision or enucleation with minimal morbidity and preservation of residual pancreatic function. Benign insulinoma has near ubiquitous dense GLP-1R expression enabling accurate localisation with radiolabelled-exendin-4 compounds (e.g. 68 Ga-NOTA-exendin-4 PET/CT), whilst the rarer and more difficult to manage metastatic insulinoma typically express SSTR and is preferably imaged with radiolabelled-SSTR analogues such as 68 Ga-DOTA-octreotate (DOTATATE) PET/CT for staging and assessment of suitability for peptide receptor radionuclide therapy (PRRT). Similar to other metastatic neuroendocrine tumours, FDG PET/CT is used in the setting of highergrade metastatic insulinoma to provide important prognostic information that can guide treatment and determine suitability for PRRT. Interestingly, these three tracers appear to represent a spectrum of differentiation, which we conceptually describe as the 'tripleflop' phenomenon, with GLP-1R > SSTR > FDG in benign insulinoma and the opposite in higher-grade disease. This paper will review the clinical syndromes of adult hypoglycaemia (including a practical overview of the differential diagnoses to be considered), comparison of techniques for insulinoma localisation with emphasis on molecular imaging before discussing its implications for management of metastatic insulinoma.

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Insulin Autoimmune Syndrome (Hirata Disease)

Cited by 76 publications

References 52 publications

A Case of Autoimmune Hypoglycemia in Switzerland

A Case of Autoimmune Hypoglycemia in Switzerland

Recurrent Hypoglycemia from Insulin Autoimmune Syndrome

Molecular imaging in the investigation of hypoglycaemic syndromes and their management

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