Insulin Autoimmune Syndrome (Hirata Disease): A Comprehensive Review Fifty Years After Its First Description

Daniele Cappellani,1 Enrico Macchia,1 Alberto Falorni,2 Piero Marchetti3 1Department of Clinical and Experimental Medicine, Unit of Endocrinology, University of Pisa, Pisa, Italy; 2Department of Medicine, Section of Internal Medicine and Endocrine and Metabolic Sciences, University of Perugia, Perug...

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Autores principales:	Cappellani D, Macchia E, Falorni A, Marchetti P
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2020
Materias:	insulin autoimmune syndrome ias hirata hypoglycemia. autoimmunity Specialties of internal medicine RC581-951
Acceso en línea:	https://doaj.org/article/31a71e4aa5d944478be5f6c8d04b3445
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spelling	oai:doaj.org-article:31a71e4aa5d944478be5f6c8d04b34452021-12-02T08:54:52ZInsulin Autoimmune Syndrome (Hirata Disease): A Comprehensive Review Fifty Years After Its First Description1178-7007https://doaj.org/article/31a71e4aa5d944478be5f6c8d04b34452020-04-01T00:00:00Zhttps://www.dovepress.com/insulin-autoimmune-syndrome-hirata-disease-a-comprehensive-review-fift-peer-reviewed-article-DMSOhttps://doaj.org/toc/1178-7007Daniele Cappellani,1 Enrico Macchia,1 Alberto Falorni,2 Piero Marchetti3 1Department of Clinical and Experimental Medicine, Unit of Endocrinology, University of Pisa, Pisa, Italy; 2Department of Medicine, Section of Internal Medicine and Endocrine and Metabolic Sciences, University of Perugia, Perugia, Italy; 3Department of Clinical and Experimental Medicine, Division of Metabolism and Cell Transplantation, University of Pisa, Pisa, ItalyCorrespondence: Daniele CappellaniDepartment of Clinical and Experimental Medicine, Unit of Endocrinology, University of Pisa, Ospedale Cisanello, via Paradisa 2, Pisa 56124, ItalyTel +39 50 995001Fax +39 50 578772Email d.cappellani@hotmail.itAbstract: Insulin autoimmune syndrome (IAS), also named Hirata’s disease, is a rare condition characterized by hypoglycemic episodes due to the presence of high titers of insulin autoantibodies (IAA). IAS is a form of immune-mediated hypoglycemia, which develops when a triggering factor (ie, a medication or a viral infection) acts on an underlying predisposing genetic background. IAS pathogenesis involves the formation of insulin-IAA complexes that induce glycemic alterations with a double-phase mechanism: IAA prevent insulin to bind its receptor in the postprandial phase, possibly resulting in mild hyperglycemia; thereafter, insulin is released from the complexes irrespective of blood glucose concentrations, thus inducing hypoglycemia. The diagnosis of IAS is challenging, requiring a careful workup aimed at excluding other causes of hyperinsulinemic hypoglycemia. The gold standard for the definitive diagnosis is the finding of IAA in a blood sample. Because IAS is frequently a self-remitting disease, its management mostly consists of supportive measures, such as dietary modifications, aimed at preventing the development of hypoglycemia. Pharmacological therapies may occasionally be necessary for patients presenting with severe manifestations of IAS. Available therapies may include drugs that reduce pancreatic insulin secretion (somatostatin analogues and diazoxide, for instance) and immunosuppressive agents (glucocorticoids, azathioprine and rituximab). The purpose of this review is to provide a comprehensive analysis of the disease, by describing the burden of knowledge that has been obtained in the 50 years following its first description, took in 1970, and by highlighting the points that are still unclear in its pathogenesis and management.Keywords: insulin autoimmune syndrome, IAS, Hirata, hypoglycemia, autoimmunityCappellani DMacchia EFalorni AMarchetti PDove Medical Pressarticleinsulin autoimmune syndromeiashiratahypoglycemia. autoimmunitySpecialties of internal medicineRC581-951ENDiabetes, Metabolic Syndrome and Obesity: Targets and Therapy, Vol Volume 13, Pp 963-978 (2020)
institution	DOAJ
collection	DOAJ
language	EN
topic	insulin autoimmune syndrome ias hirata hypoglycemia. autoimmunity Specialties of internal medicine RC581-951
spellingShingle	insulin autoimmune syndrome ias hirata hypoglycemia. autoimmunity Specialties of internal medicine RC581-951 Cappellani D Macchia E Falorni A Marchetti P Insulin Autoimmune Syndrome (Hirata Disease): A Comprehensive Review Fifty Years After Its First Description
description	Daniele Cappellani,1 Enrico Macchia,1 Alberto Falorni,2 Piero Marchetti3 1Department of Clinical and Experimental Medicine, Unit of Endocrinology, University of Pisa, Pisa, Italy; 2Department of Medicine, Section of Internal Medicine and Endocrine and Metabolic Sciences, University of Perugia, Perugia, Italy; 3Department of Clinical and Experimental Medicine, Division of Metabolism and Cell Transplantation, University of Pisa, Pisa, ItalyCorrespondence: Daniele CappellaniDepartment of Clinical and Experimental Medicine, Unit of Endocrinology, University of Pisa, Ospedale Cisanello, via Paradisa 2, Pisa 56124, ItalyTel +39 50 995001Fax +39 50 578772Email d.cappellani@hotmail.itAbstract: Insulin autoimmune syndrome (IAS), also named Hirata’s disease, is a rare condition characterized by hypoglycemic episodes due to the presence of high titers of insulin autoantibodies (IAA). IAS is a form of immune-mediated hypoglycemia, which develops when a triggering factor (ie, a medication or a viral infection) acts on an underlying predisposing genetic background. IAS pathogenesis involves the formation of insulin-IAA complexes that induce glycemic alterations with a double-phase mechanism: IAA prevent insulin to bind its receptor in the postprandial phase, possibly resulting in mild hyperglycemia; thereafter, insulin is released from the complexes irrespective of blood glucose concentrations, thus inducing hypoglycemia. The diagnosis of IAS is challenging, requiring a careful workup aimed at excluding other causes of hyperinsulinemic hypoglycemia. The gold standard for the definitive diagnosis is the finding of IAA in a blood sample. Because IAS is frequently a self-remitting disease, its management mostly consists of supportive measures, such as dietary modifications, aimed at preventing the development of hypoglycemia. Pharmacological therapies may occasionally be necessary for patients presenting with severe manifestations of IAS. Available therapies may include drugs that reduce pancreatic insulin secretion (somatostatin analogues and diazoxide, for instance) and immunosuppressive agents (glucocorticoids, azathioprine and rituximab). The purpose of this review is to provide a comprehensive analysis of the disease, by describing the burden of knowledge that has been obtained in the 50 years following its first description, took in 1970, and by highlighting the points that are still unclear in its pathogenesis and management.Keywords: insulin autoimmune syndrome, IAS, Hirata, hypoglycemia, autoimmunity
format	article
author	Cappellani D Macchia E Falorni A Marchetti P
author_facet	Cappellani D Macchia E Falorni A Marchetti P
author_sort	Cappellani D
title	Insulin Autoimmune Syndrome (Hirata Disease): A Comprehensive Review Fifty Years After Its First Description
title_short	Insulin Autoimmune Syndrome (Hirata Disease): A Comprehensive Review Fifty Years After Its First Description
title_full	Insulin Autoimmune Syndrome (Hirata Disease): A Comprehensive Review Fifty Years After Its First Description
title_fullStr	Insulin Autoimmune Syndrome (Hirata Disease): A Comprehensive Review Fifty Years After Its First Description
title_full_unstemmed	Insulin Autoimmune Syndrome (Hirata Disease): A Comprehensive Review Fifty Years After Its First Description
title_sort	insulin autoimmune syndrome (hirata disease): a comprehensive review fifty years after its first description
publisher	Dove Medical Press
publishDate	2020
url	https://doaj.org/article/31a71e4aa5d944478be5f6c8d04b3445
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Insulin Autoimmune Syndrome (Hirata Disease): A Comprehensive Review Fifty Years After Its First Description

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