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Insulin autoimmune syndrome

MedGen UID:
678528
•Concept ID:
C0854359
•
Disease or Syndrome
Synonyms: Hirata disease; insulin autoimmune hypoglycemia; insulin autoimmune syndrome
SNOMED CT: Insulin autoimmune syndrome (408539000); Hirata disease (408539000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018465
Orphanet: ORPHA411593

Definition

A rare endocrine disease characterised by hyperinsulinaemic hypoglycaemia associated with the presence of autoantibodies to endogenous insulin without previous exposure to exogenous insulin. Patients usually present in adulthood with postprandial, fasting or exercise-induced hypoglycaemia, often with pronounced neuroglycopenic symptoms. Laboratory investigations reveal markedly elevated serum insulin, as well as increased C-peptide and proinsulin. The condition may be associated with other autoimmune diseases, monoclonal gammopathy, and/or recent exposure to certain medications. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVInsulin autoimmune syndrome

Professional guidelines

PubMed

Diagnosis and treatment of anti-insulin antibody-mediated labile glycaemia in insulin-treated diabetes.
Church DS, Barker P, Burling KA, Shinwari SK, Kennedy C, Smith D, Macfarlane DP, Kernohan A, Stears A, Karamat MA, Whyte K, Narendran P, Halsall DJ, Semple RK
Diabet Med 2023 Nov;40(11):e15194. Epub 2023 Sep 1 doi: 10.1111/dme.15194. PMID: 37562398Free PMC Article
Novel management of insulin autoimmune syndrome with methylprednisolone and flash glucose monitoring.
Monteiro SS, Santos TS, Dores J
Ann Endocrinol (Paris) 2023 Feb;84(1):83-85. Epub 2022 Oct 15 doi: 10.1016/j.ando.2022.10.001. PMID: 36252848
Management of Insulin Autoimmune Hypoglycaemia: Single-centre experience from Western India with systematic review of world literature.
Patel M, Shah R, Ramteke-Jadhav S, Patil V, Patel SK, Lila A, Shah N, Bandgar T
Clin Endocrinol (Oxf) 2020 May;92(5):409-420. Epub 2020 Feb 26 doi: 10.1111/cen.14174. PMID: 32064669

Recent clinical studies

Etiology

Diagnosis and treatment of anti-insulin antibody-mediated labile glycaemia in insulin-treated diabetes.
Church DS, Barker P, Burling KA, Shinwari SK, Kennedy C, Smith D, Macfarlane DP, Kernohan A, Stears A, Karamat MA, Whyte K, Narendran P, Halsall DJ, Semple RK
Diabet Med 2023 Nov;40(11):e15194. Epub 2023 Sep 1 doi: 10.1111/dme.15194. PMID: 37562398Free PMC Article
Alpha-Lipoic Acid and Glucose Metabolism: A Comprehensive Update on Biochemical and Therapeutic Features.
Capece U, Moffa S, Improta I, Di Giuseppe G, Nista EC, Cefalo CMA, Cinti F, Pontecorvi A, Gasbarrini A, Giaccari A, Mezza T
Nutrients 2022 Dec 21;15(1) doi: 10.3390/nu15010018. PMID: 36615676Free PMC Article
Uncooked cornstarch for the prevention of hypoglycemic events.
Della Pepa G, Vetrani C, Lupoli R, Massimino E, Lembo E, Riccardi G, Capaldo B
Crit Rev Food Sci Nutr 2022;62(12):3250-3263. Epub 2021 Jan 18 doi: 10.1080/10408398.2020.1864617. PMID: 33455416
Analysis of the clinical characteristics of insulin autoimmune syndrome induced by alpha-lipoic acid.
Li Z, Su Y, Yi D, Wu C, Fang W, Wang C
J Clin Pharm Ther 2021 Oct;46(5):1295-1300. Epub 2021 Apr 5 doi: 10.1111/jcpt.13427. PMID: 33821530
Insulin Autoimmune Syndrome Diagnosis and Therapy in a Single Chinese Center.
Yuan T, Li J, Li M, Li N, Duan L, Ping F, Li W, Wang L, Zhao W
Clin Ther 2019 May;41(5):920-928. Epub 2019 Apr 13 doi: 10.1016/j.clinthera.2019.03.009. PMID: 30992145

Diagnosis

Hypoglycemia and Multivitamins.
Revankar V, Lakhpatia P, Kelkar S, Chincholkar S, Dalal K, Kewalramani K
J Assoc Physicians India 2024 Aug;72(8):99-100. doi: 10.59556/japi.72.0595. PMID: 39163080
Diagnosis and treatment of anti-insulin antibody-mediated labile glycaemia in insulin-treated diabetes.
Church DS, Barker P, Burling KA, Shinwari SK, Kennedy C, Smith D, Macfarlane DP, Kernohan A, Stears A, Karamat MA, Whyte K, Narendran P, Halsall DJ, Semple RK
Diabet Med 2023 Nov;40(11):e15194. Epub 2023 Sep 1 doi: 10.1111/dme.15194. PMID: 37562398Free PMC Article
Endogenous hyperinsulinemic hypoglycemia: case series and literature review.
Zhang C, Zhang H, Huang W
Endocrine 2023 Apr;80(1):40-46. Epub 2022 Dec 2 doi: 10.1007/s12020-022-03268-5. PMID: 36459334Free PMC Article
Alpha-Lipoic Acid and Glucose Metabolism: A Comprehensive Update on Biochemical and Therapeutic Features.
Capece U, Moffa S, Improta I, Di Giuseppe G, Nista EC, Cefalo CMA, Cinti F, Pontecorvi A, Gasbarrini A, Giaccari A, Mezza T
Nutrients 2022 Dec 21;15(1) doi: 10.3390/nu15010018. PMID: 36615676Free PMC Article
Uncooked cornstarch for the prevention of hypoglycemic events.
Della Pepa G, Vetrani C, Lupoli R, Massimino E, Lembo E, Riccardi G, Capaldo B
Crit Rev Food Sci Nutr 2022;62(12):3250-3263. Epub 2021 Jan 18 doi: 10.1080/10408398.2020.1864617. PMID: 33455416

Therapy

Hypoglycemia and Multivitamins.
Revankar V, Lakhpatia P, Kelkar S, Chincholkar S, Dalal K, Kewalramani K
J Assoc Physicians India 2024 Aug;72(8):99-100. doi: 10.59556/japi.72.0595. PMID: 39163080
Endogenous hyperinsulinemic hypoglycemia: case series and literature review.
Zhang C, Zhang H, Huang W
Endocrine 2023 Apr;80(1):40-46. Epub 2022 Dec 2 doi: 10.1007/s12020-022-03268-5. PMID: 36459334Free PMC Article
Alpha-Lipoic Acid and Glucose Metabolism: A Comprehensive Update on Biochemical and Therapeutic Features.
Capece U, Moffa S, Improta I, Di Giuseppe G, Nista EC, Cefalo CMA, Cinti F, Pontecorvi A, Gasbarrini A, Giaccari A, Mezza T
Nutrients 2022 Dec 21;15(1) doi: 10.3390/nu15010018. PMID: 36615676Free PMC Article
Uncooked cornstarch for the prevention of hypoglycemic events.
Della Pepa G, Vetrani C, Lupoli R, Massimino E, Lembo E, Riccardi G, Capaldo B
Crit Rev Food Sci Nutr 2022;62(12):3250-3263. Epub 2021 Jan 18 doi: 10.1080/10408398.2020.1864617. PMID: 33455416
Insulin Autoimmune Syndrome Diagnosis and Therapy in a Single Chinese Center.
Yuan T, Li J, Li M, Li N, Duan L, Ping F, Li W, Wang L, Zhao W
Clin Ther 2019 May;41(5):920-928. Epub 2019 Apr 13 doi: 10.1016/j.clinthera.2019.03.009. PMID: 30992145

Prognosis

A review on nondiabetic hypoglycemia from various causes: Case series report.
Gan L, Zhu X, Gao Y, Zhong M, Liao S, Huang G, Yan Y
Medicine (Baltimore) 2023 Nov 24;102(47):e36273. doi: 10.1097/MD.0000000000036273. PMID: 38013348Free PMC Article
Endogenous hyperinsulinemic hypoglycemia: case series and literature review.
Zhang C, Zhang H, Huang W
Endocrine 2023 Apr;80(1):40-46. Epub 2022 Dec 2 doi: 10.1007/s12020-022-03268-5. PMID: 36459334Free PMC Article
Insulin Autoimmune Syndrome Diagnosis and Therapy in a Single Chinese Center.
Yuan T, Li J, Li M, Li N, Duan L, Ping F, Li W, Wang L, Zhao W
Clin Ther 2019 May;41(5):920-928. Epub 2019 Apr 13 doi: 10.1016/j.clinthera.2019.03.009. PMID: 30992145
Insulin autoimmune syndrome in a pregnant female: A rare case report.
Zeng XX, Tang YL, Hu KX, Wang J, Zhu LY, Liu JY, Xu J
Medicine (Baltimore) 2017 Dec;96(51):e9213. doi: 10.1097/MD.0000000000009213. PMID: 29390469Free PMC Article
Autoimmune forms of hypoglycemia.
Lupsa BC, Chong AY, Cochran EK, Soos MA, Semple RK, Gorden P
Medicine (Baltimore) 2009 May;88(3):141-153. doi: 10.1097/MD.0b013e3181a5b42e. PMID: 19440117

Clinical prediction guides

Hypoglycemia Caused by Exogenous Insulin Antibody Syndrome: A Large Single-Center Case Series From China.
Liu Y, Ping F, Yu J, Lv L, Zhao Y, Qi M, Li W, Xu L, Yu M, Li M, Zhang H, Li Y
J Clin Endocrinol Metab 2023 Feb 15;108(3):713-717. doi: 10.1210/clinem/dgac578. PMID: 36219196
Comparison of patient characteristics between East Asian and non-East Asian patients with insulin autoimmune syndrome.
Oest L, Roden M, Müssig K
Clin Endocrinol (Oxf) 2022 Mar;96(3):328-338. Epub 2021 Nov 14 doi: 10.1111/cen.14634. PMID: 34778997
Insulin Autoimmune Syndrome: A Case of Clopidogrel-induced Autoimmune Hypoglycemia.
Calder GL, Ward GM, Sachithanandan N, MacIsaac RJ
J Clin Endocrinol Metab 2020 Apr 1;105(4) doi: 10.1210/clinem/dgz301. PMID: 32182368
Assessment and Management of Anti-Insulin Autoantibodies in Varying Presentations of Insulin Autoimmune Syndrome.
Church D, Cardoso L, Kay RG, Williams CL, Freudenthal B, Clarke C, Harris J, Moorthy M, Karra E, Gribble FM, Reimann F, Burling K, Williams AJK, Munir A, Jones TH, Führer D, Moeller LC, Cohen M, Khoo B, Halsall D, Semple RK
J Clin Endocrinol Metab 2018 Oct 1;103(10):3845-3855. doi: 10.1210/jc.2018-00972. PMID: 30085133Free PMC Article
Insulin autoimmune syndrome (Hirata Disease) in European Caucasians taking α-lipoic acid.
Gullo D, Evans JL, Sortino G, Goldfine ID, Vigneri R
Clin Endocrinol (Oxf) 2014 Aug;81(2):204-9. Epub 2013 Oct 23 doi: 10.1111/cen.12334. PMID: 24111525

Recent systematic reviews

Hypoglycemia as a potential risk for patients taking clopidogrel: A systematic review and meta-analysis.
Chen S, Qiang J, Zhang Y, Zhao B, Tian R, Yuan T, Li M, Li M, Li Y, Zhu H, Pan H
Front Endocrinol (Lausanne) 2023;14:1091933. Epub 2023 Feb 28 doi: 10.3389/fendo.2023.1091933. PMID: 36926026Free PMC Article
Management of Insulin Autoimmune Hypoglycaemia: Single-centre experience from Western India with systematic review of world literature.
Patel M, Shah R, Ramteke-Jadhav S, Patil V, Patel SK, Lila A, Shah N, Bandgar T
Clin Endocrinol (Oxf) 2020 May;92(5):409-420. Epub 2020 Feb 26 doi: 10.1111/cen.14174. PMID: 32064669

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