RARE DISEASE
Insulin autoimmune syndrome
Insulin autoimmune syndrome
Insulin autoimmune syndrome
Synonyms: Hirata disease
Synonyms: Hirata disease
Synonyms: Hirata disease
Drug discovery
1
drug
With orphan designation
Overview
Insulin Autoimmune Syndrome (IAS) is a rare endocrine disorder characterized by spontaneous hypoglycemia due to endogenous insulin autoantibodies (IAAs), which bind insulin and unpredictably release it, causing postprandial or fasting hypoglycemia. Diagnosis requires elevated serum insulin, detectable IAAs without prior insulin exposure, and exclusion of insulinoma. Most cases are self-limiting, resolving within 3–6 months, but severe episodes may necessitate immunosuppression or plasmapheresis [1][6][12].
Population
Predominantly reported in Asian populations (especially Japanese), with genetic links to HLA-DR4 alleles [2][8].
Associated with sulfhydryl-containing medications (e.g., methimazole, alpha-lipoic acid) and autoimmune conditions (e.g., Graves' disease, rheumatoid arthritis) [1][6][17].
Increasingly observed in Caucasians, often linked to drug exposure [2][8].
Burden
High morbidity from neuroglycopenic symptoms (e.g., coma, seizures) and frequent hospitalizations [1][6].
Risk of misdiagnosis as insulinoma, leading to unnecessary surgeries [6][17].
Self-limiting in 70–80% of cases, but prolonged symptoms in patients with monoclonal antibodies or comorbidities [8][12][17].
Therapies
First-line: Dietary modifications (small, frequent low-carbohydrate meals), acarbose to delay glucose absorption [3][6][13].
Pharmacological: Diazoxide or somatostatin analogs to suppress insulin secretion; glucocorticoids, rituximab, or azathioprine for refractory cases [3][8][18].
Severe cases: Plasmapheresis or immunosuppressants to reduce antibody titers [1][3][8].
Categories: rare endocrine diseases
Research Papers
176 drug discovery papers about Insulin autoimmune syndrome, with 2 first-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
176 drug discovery papers about Insulin autoimmune syndrome, with 2 first-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
categories:
Small molecules
small molecules
2026-09-30 | Hirata's Disease: A Case Report.
Insulin Autoimmune Syndrome (IAS), also known as Hirata's disease, is a rare endocrine disorder characterized by recurrent hypoglycemia due to endogenous hyperinsulinemia and the presence of insulin autoantibodies (IAAs). We report a case of IAS successfully treated with corticosteroid therapy. Notably, the definitive diagnosis was established only during the second hospitalization, after multiple recurrent hypoglycemic episodes. The delayed diagnosis reflects the nonspecific presentation and the low initial suspicion for autoimmune hypoglycemia. In the first admission, although insulinoma had been excluded, the patient was discharged without a confirmed diagnosis. This case highlights the importance of early insulin autoantibody testing in patients with unexplained endogenous hyperinsulinemic hypoglycemia to prevent diagnostic delay and unnecessary investigations. Initial evaluation prioritized exclusion of insulinoma, the most common cause of endogenous hyperinsulinemic hypoglycemia, before autoimmune testing was pursued.
2026-09-08 | Dual Thiol Drug-Induced Insulin Autoimmune Syndrome in a Non-Asian Patient Carrying HLA-DRB1*04:06: A Case Report and Literature Review.
Insulin autoimmune syndrome (IAS, Hirata's disease) is a rare cause of endogenous hyperinsulinemic hypoglycemia classically linked to high-titer insulin autoantibodies and exposure to sulfhydryl (-SH)-containing drugs. IAS is strongly HLA-associated and is most frequently reported in East Asian populations. We report a case in which simultaneous exposure to two thiol-containing agents unmasked IAS in a non-Asian patient carrying HLA-DRB1*04:06. A 60-year-old woman developed recurrent autonomic and neuroglycopenic episodes 2-4 hours after meals. Home medications included captopril and over-the-counter α-lipoic acid (ALA). During a symptomatic episode her plasma glucose was 38 mg/dL with markedly elevated serum insulin, C-peptide and proinsulin; mixed-meal testing reproduced a postprandial glucose peak followed by profound hypoglycemia accompanied by paradoxical hyperinsulinemia. Insulin autoantibodies were strongly positive (>150 U/mL) and HLA genotyping revealed DRB1*04:06. A supervised fast was non-diagnostic for insulinoma. Management comprised immediate discontinuation of both captopril and ALA (captopril replaced with losartan) and dietary measures (frequent small low-carbohydrate meals). Hypoglycemic episodes abated within two weeks and resolved by six weeks; fasting glucose, insulin and IAA titers had substantially improved by three months. To our knowledge, this is the first reported case of IAS temporally associated with concurrent captopril and ALA exposure in a non-Asian carrier of HLA-DRB1*04:06. The case highlights the need to review both prescription drugs and supplements when investigating hyperinsulinemic hypoglycemia, that IAS can occur outside typical ethnic groups when permissive HLA alleles are present, and that prompt withdrawal of offending agents with conservative measures often leads to spontaneous remission.
2026-08-28 | Alpha-lipoic acid as a trigger of insulin autoimmune syndrome: Current evidence, a case-based approach, diagnostic pitfalls, and practical management.
Insulin autoimmune syndrome (IAS), also known as Hirata disease, is a rare immune-mediated cause of endogenous hyperinsulinemic hypoglycemia characterized by circulating insulin autoantibodies (IAAs). It typically develops in individuals without prior exposure to exogenous insulin. Although IAS has been associated with viral infections and, less commonly, with autoimmune and hematologic disorders, medications and dietary supplements are considered the most frequent precipitating factors. Sulfhydryl-containing compounds are the most commonly implicated triggers. Methimazole represents the classical drug-associated cause, whereas an increasing number of cases have been attributed to the widely available antioxidant supplement alpha-lipoic acid (ALA).In genetically susceptible individuals, i.e. in those carrying the HLA alleles HLA-DRB1*04:06/04:/03/04:04, sulfhydryl-containing compounds are thought to alter insulin immunogenicity and promote the formation of IAAs. The resulting insulin-antibody complexes disrupt normal insulin kinetics, leading to recurrent hypoglycemia that may alternate with episodes of postprandial hyperglycemia. Early recognition of the syndrome is of paramount importance, as withdrawal of the triggering agent together with appropriate supportive and pharmacological management is associated with a favorable prognosis in most patients. The importance of medical nutrition therapy with frequent, small meals rich in cornstarch together with the addition of acarbose or glucocorticoids should be pointed out. This narrative review provides an overview of the current evidence regarding IAS, with particular emphasis on drug- and ALA-associated disease. We further illustrate the practical application of this evidence through the presentation of a 76-year-old man who developed IAS following ALA supplementation. To our knowledge, this is the first narrative review to comprehensively address the epidemiology, pathogenesis, diagnosis, and management of ALA-associated IAS while integrating a representative clinical case to bridge current evidence with practical clinical decision-making. By highlighting this rare but increasingly recognized cause of hypoglycemia, this work aims to enhance clinical awareness and support timely diagnosis and evidence-based management.
2026-08-01 | EP642 - ECE_3992 - The first case of insulin autoimmune syndrome (Hirata's Disease) in A Qatari female: a case report
Abstract Background Insulin Autoimmune Syndrome (IAS), also known as Hirata’s disease, is a rare cause of insulin-mediated hypoglycemia, mostly reported in East Asian populations. This condition is characterized by circulating autoantibodies that bind to endogenous insulin, creating large complexes that act as a reservoir, releasing high amounts of “free” insulin unexpectedly, causing hypoglycemia. Fewer than 10 cases have been reported in Arab nationals, and to date, no cases have been documented in Qatari national. Herein, we report the first case of IAS in a Qatari woman. Case Presentation A 39-year-old Qatari woman presenting with a three- week history of recurrent symptomatic hypoglycemia occurring approximately two weeks after discontinuing a short course of prednisolone therapy prescribed for recurrent pregnancy loss. The hypoglycemic episodes are mainly 2-4 hours after meals. Continuous glucose monitoring revealed a characteristic pattern of postprandial hyperglycemia (200-220 mg/dL) followed by severe hypoglycemia (40-50 mg/dL). She has normal liver, renal, and adrenal functions. Laboratory evaluation during a documented hypoglycemic episode (plasma glucose 59 mg/dL) revealed markedly elevated insulin levels (760 μU/mL; reference 2.6-24.9 μU/mL) and C-peptide levels (21 ng/mL; reference 1.1-4.4 ng/mL) with an insulin-to-C-peptide molar ratio of 1.1. Cross-sectional imaging excluded pancreatic lesions. Insulin autoantibody testing confirmed the diagnosis with markedly elevated titers at 84 nmol/L (reference <0.4 nmol/L). Treatment with prednisolone 20 mg daily resulted in complete symptom resolution and progressive normalization of antibody titers to 0.62 nmol/L after eight weeks of therapy. At three months post-steroids discontinuation, the patient remained free of hypoglycemia evident by normal continuous glucose monitoring. Conclusions This represents the first documented case of IAS in a Qatari national, highlighting the importance of recognizing this condition outside the traditionally affected East Asian populations to avoid unnecessary invasive diagnostic procedures or pancreatic surgery.
2026-08-01 | EP456 - ECE_2472 - Insulin autoimmune syndrome in a caucasian male: a rare cause of recurrent hypoglycemia
Abstract Insulin autoimmune syndrome (IAS), or Hirata disease, is a rare cause of hypoglycemia mediated by insulin autoantibodies (IAA). We report a 76-year-old Caucasian male with hypertension, ischemic heart disease, and benign prostatic hyperplasia who presented with recurrent syncope and seizure-like activity over two months, necessitating his third hospital admission. Initial workup, including CT/MRI brain, EEG, echocardiogram, and Holter monitoring, was unremarkable. During the third admission, hypoglycemia was confirmed (serum glucose 2.2 mmol/L), with markedly elevated insulin (>6945 pmol/L), C-peptide (2.48 nmol/L), and IAA titers (75%, normal <5.5%). Pancreatic imaging and endoscopic ultrasound excluded neuroendocrine tumours, and negative sulfonylurea screen confirmed IAS. The patient received intravenous hydrocortisone for two days, followed by oral prednisolone, dietary counselling for frequent low-carbohydrate meals, and continuous glucose monitoring. Rituximab was initially considered for severe, refractory hypoglycemia but was not administered due to rapid clinical and serological improvement. Follow-up revealed declining IAA titers and no further hypoglycemic episodes by five months. Historically prevalent in Japan (0.017/100 000), IAS is associated with HLA-DR4 (DRB1*0406) and triggers including sulphydryl drugs or viral infections. Rising global cases among Caucasians likely reflect increased medication use and wider IAA assay availability. IAS involves high-capacity, low-affinity IAA causing glycemic fluctuations through insulin- IAA complex dynamics. Symptoms range from mild to severe (seizures, syncope). Management is complex given an 82% spontaneous remission rate. This case highlights recognising IAS in non-Asian populations and demonstrates the efficacy of corticosteroids and dietary interventions. Recurrence is rare (<5%).
cell therapies
2022-07-12 | Islet Biology During COVID-19: Progress and Perspectives.
The coronavirus-2019 (COVID-19) pandemic has had significant impact on research directions and productivity in the past 2 years. Despite these challenges, since 2020, more than 2,500 peer-reviewed articles have been published on pancreatic islet biology. These include updates on the roles of isocitrate dehydrogenase, pyruvate kinase and incretin hormones in insulin secretion, as well as the discovery of inceptor and signalling by circulating RNAs. The year 2020 also brought advancements in in vivo and in vitro models, including a new transgenic mouse for assessing beta-cell proliferation, a "pancreas-on-a-chip" to study glucose-stimulated insulin secretion and successful genetic editing of primary human islet cells. Islet biologists evaluated the functionality of stem-cell-derived islet-like cells coated with semipermeable biomaterials to prevent autoimmune attack, revealing the importance of cell maturation after transplantation. Prompted by observations that COVID-19 symptoms can worsen for people with obesity or diabetes, researchers examined how islets are directly affected by severe acute respiratory syndrome coronavirus 2. Herein, we highlight novel functional insights, technologies and therapeutic approaches that emerged between March 2020 and July 2021, written for both scientific and lay audiences. We also include a response to these advancements from patient stakeholders, to help lend a broader perspective to developments and challenges in islet research. La pandémie de la maladie à coronavirus 2019 (COVID-19) a eu des répercussions importantes sur les orientations et la productivité de la recherche au cours de la dernière année. En dépit de ces enjeux, depuis 2020, plus de 2500 articles revus par les pairs sur la biologie des îlots pancréatiques ont été publiés, y compris des articles qui portent sur les rôles de l’isocitrate-déshydrogénase, de la pyruvate kinase et des incrétines dans la sécrétion de l’insuline, ainsi que sur la découverte de l’« inceptor » et de la signalisation par les ARN circulants. L’année 2020 a aussi donné lieu à des avancées des modèles in vivo et in vitro, notamment la nouvelle souris transgénique pour permettre l’évaluation de la prolifération de cellules bêta, le « pancréas sur puce » pour étudier la sécrétion d’insuline stimulée par le glucose et la réussite de l’édition génomique des principales cellules humaines d’îlots pancréatiques. Les biologistes spécialisés dans le domaine des îlots pancréatiques ont évalué la fonctionnalité des cellules de type îlots pancréatiques issues de cellules souches recouvertes de biomatériaux semi-perméables pour prévenir les attaques auto-immunes, révélatrices de l’importance de la maturation cellulaire après la transplantation. Du fait que l’aggravation des symptômes de la COVID-19 est observée chez les personnes obèses ou diabétiques, les chercheurs ont examiné la façon avec laquelle les îlots sont directement affectés par le coronavirus 2 du syndrome respiratoire aigu. Dans le présent article, nous présentons les nouvelles perspectives fonctionnelles, les technologies et les approches thérapeutiques qui sont apparues entre mars 2020 et juillet 2021, et destinées aux scientifiques et aux non-scientifiques. Nous proposons aussi une réponse à ces avancées issues des groupes de patients pour permettre de donner une perspective plus large aux développements et aux enjeux de la recherche sur les îlots pancréatiques.
2021-10-26 | [Insulin autoimmune syndrome : A rare, but important differential diagnosis of hypoglycemia].
A 69-year-old female patient was referred to the Medical University of Hanover for further diagnostic evaluation of recurrent severe hypoglycemia. The patient had previously been started on clopidogrel after arterial stenting for peripheral arterial obstructive disease (PAOD). The presence of an insulinoma and paraneoplastic syndrome was excluded. Increased serum insulin and insulin autoantibodies levels were confirmed, despite normal to low blood sugar levels. An insulin autoimmune syndrome was diagnosed, most likely induced by the prior intake of clopidogrel. Treatment with immunoadsorption was initiated, achieving a significant reduction in hypoglycemic events and a lasting response to treatment over 3 months.Eine 69-jährige Patientin mit rezidivierenden schweren Hypoglykämien wurde zur weiteren Diagnostik der Medizinischen Hochschule Hannover zugewiesen. Zuvor hatte die Patientin nach einem Stenting, das aufgrund einer peripheren arteriellen Verschlusskrankheit (pAVK) erforderlich war, mit einer Clopidogreleinnahme begonnen. Das Vorliegen eines Insulinoms und eines paraneoplastischen Syndroms wurde ausgeschlossen. Trotz niedrig normaler Blutzuckerkonzentrationen fanden sich erhöhte Insulin- und Insulinautoantikörperkonzentrationen. Diagnostiziert wurde ein Insulinautoimmunsyndrom, a.e. (am ehesten) ausgelöst durch vorausgegangene Clopidogreleinnahme. Eine Behandlung mithilfe der Immunadsorption wurde initiiert; diese erzielte eine signifikante Reduktion der hypoglykämischen Ereignisse und ein anhaltendes Therapieansprechen über 3 Monate.
2021-01-04 | Insulin Autoimmune Syndrome: A Case of Clopidogrel-induced Autoimmune Hypoglycemia.
Insulin autoimmune syndrome (IAS) is characterized by hyperinsulinemic hypoglycemia with elevated anti-insulin antibodies. Most commonly observed in the Japanese population, elsewhere it is rare and associated with autoimmune diseases, plasma cell dyscrasias, or sulfhydryl group medications. The active metabolite of clopidogrel has a sulfhydryl group and here we report a case of clopidogrel-induced IAS. A 67-year-old man was admitted with severe hyperinsulinemic hypoglycemia requiring continuous intravenous infusion of 10% dextrose to sustain euglycemia. His symptoms of hypoglycemia had started after commencing dual antiplatelet therapy (including clopidogrel) for ischemic heart disease 9 months earlier. The hypoglycemia was associated with elevated insulin, proinsulin, c-peptide, and anti-insulin antibody titers as well as the HLA-DRB1*04 haplotype. Multiple localizing studies were negative for an insulinoma. A diagnosis of IAS was thus made. Clopidogrel cessation, oral dexamethasone, and diazoxide therapy were not sufficient to safely wean the dextrose infusion. Plasma exchange was ultimately effective. This case highlights a case of severe IAS. Given the ubiquity of clopidogrel, IAS should be remembered as a rare adverse effect.
2020-12-09 | A combination of plasma exchange and steroids in the treatment of a-lipoic acid-induced insulin autoimmune syndrome
Not required for Clinical Vignette.
2020-02-22 | Insulin Autoimmune Syndrome Treated with Plasmapharesis
A 66-year male with a history of Central Serous Retinopathy presented with recurrent episodes of hypoglycemia. On evaluation, he was found to have insulin-mediated hypoglycemia with serum insulin of 300uIU/ml, C peptide 27.51ng/ml, when the blood glucose was 46mg/dl. High insulin levels above 100uIU/ml, led to suspicion of Autoimmune hypoglycemia and were confirmed by a high anti-insulin antibody titer of 300U/ml. Imaging was negative for Insulinoma. The patient was started on low dose oral prednisolone under ophthalmological monitoring, but as there was no symptomatic improvement, the dose was increased following which there was a flare-up of CSR. The patient was initiated on plasmapheresis following which his hypoglycemia improved with drop in anti-insulin antibody titers to 29U/ml. The patient was maintained on low dose steroids, which were tapered and stopped over the next six months with complete resolution of hypoglycemia and normalization of anti-insulin antibody titers.
antibodies
2026-08-01 | P196 - ECE_3434 - Sustained remission of severe insulin autoimmune syndrome following rituximab therapy: a two-year follow-up case report
Abstract Background Insulin Autoimmune Syndrome (IAS), or Hirata disease, is a rare but important cause of endogenous hyperinsulinemic hypoglycemia. It is characterized by the triad of spontaneous hypoglycemia, inappropriately elevated insulin and C-peptide levels, and the presence of insulin autoantibodies (IAA), in the absence of exogenous insulin use. While many cases are self-limiting, severe or steroid-refractory presentations may necessitate immunosuppressive treatment. Case Series Case 1: A 28-year-old woman presented with recurrent episodes of loss of consciousness and adrenergic/neuroglycopenic symptoms. Point-of-care glucose was 30 mg/dL. Biochemistry revealed insulin >3000 μU/mL and C-peptide 7.1 ng/mL. Imaging (MRI, EUS, Ga-68 DOTATATE PET/CT) was negative for insulinoma. Strongly positive insulin autoantibodies (>175 U/mL) confirmed IAS. Initial treatment with high-dose diazoxide and steroids was poorly tolerated. Rituximab (2 × 1 g one week apart) led to rapid and sustained remission: insulin declined to 958 μU/mL at 1 month and 110 μU/mL at 28 months. CD19/CD20+ cells were fully depleted by 3 months. The patient remained asymptomatic with dietary management and discontinued all medications. Genetic testing revealed HLA-DRB1*0406 and a heterozygous MAPK8IP1 variant. Case 2: A 79-year-old man was hospitalized with recurrent hypoglycemia and documented Whipple’s triad. Fasting glucose was 38 mg/dL, with C-peptide 33.27 ng/mL and insulin >1000 μU/mL. Imaging (MRI, octreoscan) and selective arterial calcium injection (SACI) testing showed no pancreatic lesion or lateralization. IAA were positive (2.6 U/mL; ref <1.1), confirming IAS. He had a history of polymyalgia rheumatica. Diazoxide was initially prescribed but stopped due to edema and GI intolerance. Prednisolone (40 mg) improved hypoglycemia but caused disabling myopathy. Rituximab was administered with complete resolution of hypoglycemia and disappearance of IAA at 1-year post-treatment. Conclusions These cases illustrate the diagnostic challenges of IAS and the limitations of standard therapy. Rituximab achieved sustained remission in both patients with steroid-refractory IAS, irrespective of age or autoimmune background. B-cell depletion may represent a valuable treatment option in selected severe cases. Long-term follow-up with biochemical monitoring is essential to assess durability of response.
2026-05-29 | Refractory severe type B insulin resistance treated with daratumumab.
Type B insulin resistance syndrome (TBIRS) is a rare autoimmune condition characterized by insulin receptor autoantibodies, often in association with systemic lupus erythematosus (SLE). It can cause extreme insulin resistance, with fewer than 200 cases described worldwide. A 24-year-old man with no prior autoimmune history presented with diabetic ketoacidosis and severe insulin resistance, requiring up to 215 000 units of insulin daily. Laboratory studies revealed high-titer antinuclear antibodies, hypocomplementemia, lupus-specific autoantibodies, and mesangial proliferative lupus nephritis, confirming new-onset SLE. His management was complicated by latent tuberculosis, neutropenia, and cyclophosphamide-induced hearing loss. Despite intensive therapy with corticosteroids, rituximab, cyclophosphamide, obinutuzumab, plasmapheresis, and intravenous immunoglobulin, he remained profoundly insulin resistant, requiring very high daily insulin doses, with persistently elevated insulin receptor antibody titers. Initiation of daratumumab, a CD38 monoclonal antibody, resulted in marked clinical improvement, with progressive decline in insulin requirements and eventual discontinuation of insulin 17 months after presentation. He achieved durable remission with normalization of hemoglobin A1c. This unusually severe case of TBIRS highlights the limitations of conventional B-cell-directed therapies, where long-lived plasma cells sustain pathogenic autoantibodies. Plasma cell-targeted strategies such as daratumumab may represent an effective therapeutic option in refractory cases.
2026-03-13 | Clinical Characteristics and Risk Factors of Exogenous Insulin Antibody Syndrome in Patients with Diabetes: A Retrospective Cross-Sectional Study.
Insulin autoimmune syndrome (IAS) is a rare hypoglycemic disorder often confused with insulinoma or insulin overdose. Patients with diabetes on insulin therapy increasingly show insulin autoantibodies (IAAs), presenting symptoms similar to classic IAS, termed exogenous insulin antibody syndrome (EIAS). This study examines EIAS clinical features and risk factors. Patients with diabetes with IAA test results, admitted to our hospital between June 2023 and March 2024 were retrospectively enrolled. Participants were stratified into control and EIAS groups on the basis of IAA status. Clinical characteristics were compared between groups, independent risk factors for EIAS were identified by multivariate logistic regression, and the diagnostic utility of fasting insulin for predicting EIAS was assessed with receiver-operating-characteristic (ROC) curve analysis. Of 120 patients with diabetes and available IAAs results, 37 met criteria for EIAS. Compared with controls, EIAS patients were older, had longer diabetes duration, were more often treated with insulin aspart or premixed human insulin, and received higher daily insulin doses. Paradoxically, EIAS patients had markedly lower levels of fasting blood glucose and HbA1c, while higher fasting and 2-h post-prandial insulin concentrations, as well as HOMA-IR. Multivariate logistic regression analysis showed that elevated fasting insulin levels were independently associated with increased risk of EIAS. For every 1 uU/mL increase in fasting insulin, the risk of EIAS increased by 3% (OR = 1.03, 95% CI: 1.00-1.05). The fasting insulin level demonstrated high overall diagnostic and predictive efficacy for EIAS, with an area under the curve (AUC) of 0.782 (95% CI: 0.691-0.872). The optimal diagnostic cutoff value was 6.975 uU/mL, with a sensitivity of 73.0% and a specificity of 81.9%. EIAS patients were identified with advanced age, prolonged diabetes duration, high insulin dosage, hypoglycemia, and hyperinsulinemia. Fasting insulin level is independently associated with EIAS risk and demonstrates good diagnostic performance.
2026-02-05 | Three Interesting Cases of Hypoglycemia: A Case Series.
Hypoglycemia can be due to exogenous hyperinsulinemic hypoglycemia or endogenous hyperinsulinemic hypoglycemia (ENHH). ENHH is a rare entity characterized by inappropriately elevated plasma insulin in the background of low plasma glucose levels. ENHH, being an uncommon disease, is caused by many rare entities, with drug-induced being the most common cause of hypoglycemia. Other causes include insulinoma, insulin autoimmune syndrome, and nesidioblastosis. Here, we report three rare cases of non-diabetic hypoglycemia referred to the endocrinology clinic who had signs and symptoms of hypoglycemia fulfilling Whipple's triad with evidence of hyperinsulinemia despite low plasma glucose. All the appropriate biochemical investigations along with imaging studies were conducted to find out the etiology of hypoglycemia. After taking detailed medical history and clinical examination with biochemical and radiological investigations, patients were diagnosed with insulin autoimmune syndrome, insulinoma, and drug-induced hypoglycemia. Appropriate management was started, and patients were relieved of hypoglycemia. Diseases causing hypoglycemia have similar manifestation, but the diagnosis and management are always a challenging task. Hence, an accurate and early diagnosis is always a deciding factor for further prognosis.
2026-01-01 | Case Report on Hypoglycemia – Beyond Diet, Drugs and Diabetes
Hypoglycemia is a common presentation for various conditions presenting to the emergency department. After ruling out drugs as a cause, especially in nondiabetic individuals, classification to hyperinsulinemic or non-hyper insulinemic hypoglycemia is the next step. Since insulinoma is one of the most common causes of hyperinsulinemia, several imaging modalities are typically used in the search for it. However, in the presence of autoimmune autoantibody syndrome should be thought of. Here we report a case of an elderly non diabetic female with recurrent hypoglycemia. She was noticed to have late postprandial hypoglycemia. Investigation revealed raised insulin levels, raised C peptide levels, and high titers of insulin auto-antibodies. The patient was diagnosed with Insulin antibody syndrome (IAS). IAS may not be a rare syndrome; physicians need to be more vigilant to diagnose and manage such patients.
small molecules
2026-09-30 | Hirata's Disease: A Case Report.
Insulin Autoimmune Syndrome (IAS), also known as Hirata's disease, is a rare endocrine disorder characterized by recurrent hypoglycemia due to endogenous hyperinsulinemia and the presence of insulin autoantibodies (IAAs). We report a case of IAS successfully treated with corticosteroid therapy. Notably, the definitive diagnosis was established only during the second hospitalization, after multiple recurrent hypoglycemic episodes. The delayed diagnosis reflects the nonspecific presentation and the low initial suspicion for autoimmune hypoglycemia. In the first admission, although insulinoma had been excluded, the patient was discharged without a confirmed diagnosis. This case highlights the importance of early insulin autoantibody testing in patients with unexplained endogenous hyperinsulinemic hypoglycemia to prevent diagnostic delay and unnecessary investigations. Initial evaluation prioritized exclusion of insulinoma, the most common cause of endogenous hyperinsulinemic hypoglycemia, before autoimmune testing was pursued.
2026-09-08 | Dual Thiol Drug-Induced Insulin Autoimmune Syndrome in a Non-Asian Patient Carrying HLA-DRB1*04:06: A Case Report and Literature Review.
Insulin autoimmune syndrome (IAS, Hirata's disease) is a rare cause of endogenous hyperinsulinemic hypoglycemia classically linked to high-titer insulin autoantibodies and exposure to sulfhydryl (-SH)-containing drugs. IAS is strongly HLA-associated and is most frequently reported in East Asian populations. We report a case in which simultaneous exposure to two thiol-containing agents unmasked IAS in a non-Asian patient carrying HLA-DRB1*04:06. A 60-year-old woman developed recurrent autonomic and neuroglycopenic episodes 2-4 hours after meals. Home medications included captopril and over-the-counter α-lipoic acid (ALA). During a symptomatic episode her plasma glucose was 38 mg/dL with markedly elevated serum insulin, C-peptide and proinsulin; mixed-meal testing reproduced a postprandial glucose peak followed by profound hypoglycemia accompanied by paradoxical hyperinsulinemia. Insulin autoantibodies were strongly positive (>150 U/mL) and HLA genotyping revealed DRB1*04:06. A supervised fast was non-diagnostic for insulinoma. Management comprised immediate discontinuation of both captopril and ALA (captopril replaced with losartan) and dietary measures (frequent small low-carbohydrate meals). Hypoglycemic episodes abated within two weeks and resolved by six weeks; fasting glucose, insulin and IAA titers had substantially improved by three months. To our knowledge, this is the first reported case of IAS temporally associated with concurrent captopril and ALA exposure in a non-Asian carrier of HLA-DRB1*04:06. The case highlights the need to review both prescription drugs and supplements when investigating hyperinsulinemic hypoglycemia, that IAS can occur outside typical ethnic groups when permissive HLA alleles are present, and that prompt withdrawal of offending agents with conservative measures often leads to spontaneous remission.
2026-08-28 | Alpha-lipoic acid as a trigger of insulin autoimmune syndrome: Current evidence, a case-based approach, diagnostic pitfalls, and practical management.
Insulin autoimmune syndrome (IAS), also known as Hirata disease, is a rare immune-mediated cause of endogenous hyperinsulinemic hypoglycemia characterized by circulating insulin autoantibodies (IAAs). It typically develops in individuals without prior exposure to exogenous insulin. Although IAS has been associated with viral infections and, less commonly, with autoimmune and hematologic disorders, medications and dietary supplements are considered the most frequent precipitating factors. Sulfhydryl-containing compounds are the most commonly implicated triggers. Methimazole represents the classical drug-associated cause, whereas an increasing number of cases have been attributed to the widely available antioxidant supplement alpha-lipoic acid (ALA).In genetically susceptible individuals, i.e. in those carrying the HLA alleles HLA-DRB1*04:06/04:/03/04:04, sulfhydryl-containing compounds are thought to alter insulin immunogenicity and promote the formation of IAAs. The resulting insulin-antibody complexes disrupt normal insulin kinetics, leading to recurrent hypoglycemia that may alternate with episodes of postprandial hyperglycemia. Early recognition of the syndrome is of paramount importance, as withdrawal of the triggering agent together with appropriate supportive and pharmacological management is associated with a favorable prognosis in most patients. The importance of medical nutrition therapy with frequent, small meals rich in cornstarch together with the addition of acarbose or glucocorticoids should be pointed out. This narrative review provides an overview of the current evidence regarding IAS, with particular emphasis on drug- and ALA-associated disease. We further illustrate the practical application of this evidence through the presentation of a 76-year-old man who developed IAS following ALA supplementation. To our knowledge, this is the first narrative review to comprehensively address the epidemiology, pathogenesis, diagnosis, and management of ALA-associated IAS while integrating a representative clinical case to bridge current evidence with practical clinical decision-making. By highlighting this rare but increasingly recognized cause of hypoglycemia, this work aims to enhance clinical awareness and support timely diagnosis and evidence-based management.
2026-08-01 | EP642 - ECE_3992 - The first case of insulin autoimmune syndrome (Hirata's Disease) in A Qatari female: a case report
Abstract Background Insulin Autoimmune Syndrome (IAS), also known as Hirata’s disease, is a rare cause of insulin-mediated hypoglycemia, mostly reported in East Asian populations. This condition is characterized by circulating autoantibodies that bind to endogenous insulin, creating large complexes that act as a reservoir, releasing high amounts of “free” insulin unexpectedly, causing hypoglycemia. Fewer than 10 cases have been reported in Arab nationals, and to date, no cases have been documented in Qatari national. Herein, we report the first case of IAS in a Qatari woman. Case Presentation A 39-year-old Qatari woman presenting with a three- week history of recurrent symptomatic hypoglycemia occurring approximately two weeks after discontinuing a short course of prednisolone therapy prescribed for recurrent pregnancy loss. The hypoglycemic episodes are mainly 2-4 hours after meals. Continuous glucose monitoring revealed a characteristic pattern of postprandial hyperglycemia (200-220 mg/dL) followed by severe hypoglycemia (40-50 mg/dL). She has normal liver, renal, and adrenal functions. Laboratory evaluation during a documented hypoglycemic episode (plasma glucose 59 mg/dL) revealed markedly elevated insulin levels (760 μU/mL; reference 2.6-24.9 μU/mL) and C-peptide levels (21 ng/mL; reference 1.1-4.4 ng/mL) with an insulin-to-C-peptide molar ratio of 1.1. Cross-sectional imaging excluded pancreatic lesions. Insulin autoantibody testing confirmed the diagnosis with markedly elevated titers at 84 nmol/L (reference <0.4 nmol/L). Treatment with prednisolone 20 mg daily resulted in complete symptom resolution and progressive normalization of antibody titers to 0.62 nmol/L after eight weeks of therapy. At three months post-steroids discontinuation, the patient remained free of hypoglycemia evident by normal continuous glucose monitoring. Conclusions This represents the first documented case of IAS in a Qatari national, highlighting the importance of recognizing this condition outside the traditionally affected East Asian populations to avoid unnecessary invasive diagnostic procedures or pancreatic surgery.
2026-08-01 | EP456 - ECE_2472 - Insulin autoimmune syndrome in a caucasian male: a rare cause of recurrent hypoglycemia
Abstract Insulin autoimmune syndrome (IAS), or Hirata disease, is a rare cause of hypoglycemia mediated by insulin autoantibodies (IAA). We report a 76-year-old Caucasian male with hypertension, ischemic heart disease, and benign prostatic hyperplasia who presented with recurrent syncope and seizure-like activity over two months, necessitating his third hospital admission. Initial workup, including CT/MRI brain, EEG, echocardiogram, and Holter monitoring, was unremarkable. During the third admission, hypoglycemia was confirmed (serum glucose 2.2 mmol/L), with markedly elevated insulin (>6945 pmol/L), C-peptide (2.48 nmol/L), and IAA titers (75%, normal <5.5%). Pancreatic imaging and endoscopic ultrasound excluded neuroendocrine tumours, and negative sulfonylurea screen confirmed IAS. The patient received intravenous hydrocortisone for two days, followed by oral prednisolone, dietary counselling for frequent low-carbohydrate meals, and continuous glucose monitoring. Rituximab was initially considered for severe, refractory hypoglycemia but was not administered due to rapid clinical and serological improvement. Follow-up revealed declining IAA titers and no further hypoglycemic episodes by five months. Historically prevalent in Japan (0.017/100 000), IAS is associated with HLA-DR4 (DRB1*0406) and triggers including sulphydryl drugs or viral infections. Rising global cases among Caucasians likely reflect increased medication use and wider IAA assay availability. IAS involves high-capacity, low-affinity IAA causing glycemic fluctuations through insulin- IAA complex dynamics. Symptoms range from mild to severe (seizures, syncope). Management is complex given an 82% spontaneous remission rate. This case highlights recognising IAS in non-Asian populations and demonstrates the efficacy of corticosteroids and dietary interventions. Recurrence is rare (<5%).
cell therapies
2022-07-12 | Islet Biology During COVID-19: Progress and Perspectives.
The coronavirus-2019 (COVID-19) pandemic has had significant impact on research directions and productivity in the past 2 years. Despite these challenges, since 2020, more than 2,500 peer-reviewed articles have been published on pancreatic islet biology. These include updates on the roles of isocitrate dehydrogenase, pyruvate kinase and incretin hormones in insulin secretion, as well as the discovery of inceptor and signalling by circulating RNAs. The year 2020 also brought advancements in in vivo and in vitro models, including a new transgenic mouse for assessing beta-cell proliferation, a "pancreas-on-a-chip" to study glucose-stimulated insulin secretion and successful genetic editing of primary human islet cells. Islet biologists evaluated the functionality of stem-cell-derived islet-like cells coated with semipermeable biomaterials to prevent autoimmune attack, revealing the importance of cell maturation after transplantation. Prompted by observations that COVID-19 symptoms can worsen for people with obesity or diabetes, researchers examined how islets are directly affected by severe acute respiratory syndrome coronavirus 2. Herein, we highlight novel functional insights, technologies and therapeutic approaches that emerged between March 2020 and July 2021, written for both scientific and lay audiences. We also include a response to these advancements from patient stakeholders, to help lend a broader perspective to developments and challenges in islet research. La pandémie de la maladie à coronavirus 2019 (COVID-19) a eu des répercussions importantes sur les orientations et la productivité de la recherche au cours de la dernière année. En dépit de ces enjeux, depuis 2020, plus de 2500 articles revus par les pairs sur la biologie des îlots pancréatiques ont été publiés, y compris des articles qui portent sur les rôles de l’isocitrate-déshydrogénase, de la pyruvate kinase et des incrétines dans la sécrétion de l’insuline, ainsi que sur la découverte de l’« inceptor » et de la signalisation par les ARN circulants. L’année 2020 a aussi donné lieu à des avancées des modèles in vivo et in vitro, notamment la nouvelle souris transgénique pour permettre l’évaluation de la prolifération de cellules bêta, le « pancréas sur puce » pour étudier la sécrétion d’insuline stimulée par le glucose et la réussite de l’édition génomique des principales cellules humaines d’îlots pancréatiques. Les biologistes spécialisés dans le domaine des îlots pancréatiques ont évalué la fonctionnalité des cellules de type îlots pancréatiques issues de cellules souches recouvertes de biomatériaux semi-perméables pour prévenir les attaques auto-immunes, révélatrices de l’importance de la maturation cellulaire après la transplantation. Du fait que l’aggravation des symptômes de la COVID-19 est observée chez les personnes obèses ou diabétiques, les chercheurs ont examiné la façon avec laquelle les îlots sont directement affectés par le coronavirus 2 du syndrome respiratoire aigu. Dans le présent article, nous présentons les nouvelles perspectives fonctionnelles, les technologies et les approches thérapeutiques qui sont apparues entre mars 2020 et juillet 2021, et destinées aux scientifiques et aux non-scientifiques. Nous proposons aussi une réponse à ces avancées issues des groupes de patients pour permettre de donner une perspective plus large aux développements et aux enjeux de la recherche sur les îlots pancréatiques.
2021-10-26 | [Insulin autoimmune syndrome : A rare, but important differential diagnosis of hypoglycemia].
A 69-year-old female patient was referred to the Medical University of Hanover for further diagnostic evaluation of recurrent severe hypoglycemia. The patient had previously been started on clopidogrel after arterial stenting for peripheral arterial obstructive disease (PAOD). The presence of an insulinoma and paraneoplastic syndrome was excluded. Increased serum insulin and insulin autoantibodies levels were confirmed, despite normal to low blood sugar levels. An insulin autoimmune syndrome was diagnosed, most likely induced by the prior intake of clopidogrel. Treatment with immunoadsorption was initiated, achieving a significant reduction in hypoglycemic events and a lasting response to treatment over 3 months.Eine 69-jährige Patientin mit rezidivierenden schweren Hypoglykämien wurde zur weiteren Diagnostik der Medizinischen Hochschule Hannover zugewiesen. Zuvor hatte die Patientin nach einem Stenting, das aufgrund einer peripheren arteriellen Verschlusskrankheit (pAVK) erforderlich war, mit einer Clopidogreleinnahme begonnen. Das Vorliegen eines Insulinoms und eines paraneoplastischen Syndroms wurde ausgeschlossen. Trotz niedrig normaler Blutzuckerkonzentrationen fanden sich erhöhte Insulin- und Insulinautoantikörperkonzentrationen. Diagnostiziert wurde ein Insulinautoimmunsyndrom, a.e. (am ehesten) ausgelöst durch vorausgegangene Clopidogreleinnahme. Eine Behandlung mithilfe der Immunadsorption wurde initiiert; diese erzielte eine signifikante Reduktion der hypoglykämischen Ereignisse und ein anhaltendes Therapieansprechen über 3 Monate.
2021-01-04 | Insulin Autoimmune Syndrome: A Case of Clopidogrel-induced Autoimmune Hypoglycemia.
Insulin autoimmune syndrome (IAS) is characterized by hyperinsulinemic hypoglycemia with elevated anti-insulin antibodies. Most commonly observed in the Japanese population, elsewhere it is rare and associated with autoimmune diseases, plasma cell dyscrasias, or sulfhydryl group medications. The active metabolite of clopidogrel has a sulfhydryl group and here we report a case of clopidogrel-induced IAS. A 67-year-old man was admitted with severe hyperinsulinemic hypoglycemia requiring continuous intravenous infusion of 10% dextrose to sustain euglycemia. His symptoms of hypoglycemia had started after commencing dual antiplatelet therapy (including clopidogrel) for ischemic heart disease 9 months earlier. The hypoglycemia was associated with elevated insulin, proinsulin, c-peptide, and anti-insulin antibody titers as well as the HLA-DRB1*04 haplotype. Multiple localizing studies were negative for an insulinoma. A diagnosis of IAS was thus made. Clopidogrel cessation, oral dexamethasone, and diazoxide therapy were not sufficient to safely wean the dextrose infusion. Plasma exchange was ultimately effective. This case highlights a case of severe IAS. Given the ubiquity of clopidogrel, IAS should be remembered as a rare adverse effect.
2020-12-09 | A combination of plasma exchange and steroids in the treatment of a-lipoic acid-induced insulin autoimmune syndrome
Not required for Clinical Vignette.
2020-02-22 | Insulin Autoimmune Syndrome Treated with Plasmapharesis
A 66-year male with a history of Central Serous Retinopathy presented with recurrent episodes of hypoglycemia. On evaluation, he was found to have insulin-mediated hypoglycemia with serum insulin of 300uIU/ml, C peptide 27.51ng/ml, when the blood glucose was 46mg/dl. High insulin levels above 100uIU/ml, led to suspicion of Autoimmune hypoglycemia and were confirmed by a high anti-insulin antibody titer of 300U/ml. Imaging was negative for Insulinoma. The patient was started on low dose oral prednisolone under ophthalmological monitoring, but as there was no symptomatic improvement, the dose was increased following which there was a flare-up of CSR. The patient was initiated on plasmapheresis following which his hypoglycemia improved with drop in anti-insulin antibody titers to 29U/ml. The patient was maintained on low dose steroids, which were tapered and stopped over the next six months with complete resolution of hypoglycemia and normalization of anti-insulin antibody titers.
antibodies
2026-08-01 | P196 - ECE_3434 - Sustained remission of severe insulin autoimmune syndrome following rituximab therapy: a two-year follow-up case report
Abstract Background Insulin Autoimmune Syndrome (IAS), or Hirata disease, is a rare but important cause of endogenous hyperinsulinemic hypoglycemia. It is characterized by the triad of spontaneous hypoglycemia, inappropriately elevated insulin and C-peptide levels, and the presence of insulin autoantibodies (IAA), in the absence of exogenous insulin use. While many cases are self-limiting, severe or steroid-refractory presentations may necessitate immunosuppressive treatment. Case Series Case 1: A 28-year-old woman presented with recurrent episodes of loss of consciousness and adrenergic/neuroglycopenic symptoms. Point-of-care glucose was 30 mg/dL. Biochemistry revealed insulin >3000 μU/mL and C-peptide 7.1 ng/mL. Imaging (MRI, EUS, Ga-68 DOTATATE PET/CT) was negative for insulinoma. Strongly positive insulin autoantibodies (>175 U/mL) confirmed IAS. Initial treatment with high-dose diazoxide and steroids was poorly tolerated. Rituximab (2 × 1 g one week apart) led to rapid and sustained remission: insulin declined to 958 μU/mL at 1 month and 110 μU/mL at 28 months. CD19/CD20+ cells were fully depleted by 3 months. The patient remained asymptomatic with dietary management and discontinued all medications. Genetic testing revealed HLA-DRB1*0406 and a heterozygous MAPK8IP1 variant. Case 2: A 79-year-old man was hospitalized with recurrent hypoglycemia and documented Whipple’s triad. Fasting glucose was 38 mg/dL, with C-peptide 33.27 ng/mL and insulin >1000 μU/mL. Imaging (MRI, octreoscan) and selective arterial calcium injection (SACI) testing showed no pancreatic lesion or lateralization. IAA were positive (2.6 U/mL; ref <1.1), confirming IAS. He had a history of polymyalgia rheumatica. Diazoxide was initially prescribed but stopped due to edema and GI intolerance. Prednisolone (40 mg) improved hypoglycemia but caused disabling myopathy. Rituximab was administered with complete resolution of hypoglycemia and disappearance of IAA at 1-year post-treatment. Conclusions These cases illustrate the diagnostic challenges of IAS and the limitations of standard therapy. Rituximab achieved sustained remission in both patients with steroid-refractory IAS, irrespective of age or autoimmune background. B-cell depletion may represent a valuable treatment option in selected severe cases. Long-term follow-up with biochemical monitoring is essential to assess durability of response.
2026-05-29 | Refractory severe type B insulin resistance treated with daratumumab.
Type B insulin resistance syndrome (TBIRS) is a rare autoimmune condition characterized by insulin receptor autoantibodies, often in association with systemic lupus erythematosus (SLE). It can cause extreme insulin resistance, with fewer than 200 cases described worldwide. A 24-year-old man with no prior autoimmune history presented with diabetic ketoacidosis and severe insulin resistance, requiring up to 215 000 units of insulin daily. Laboratory studies revealed high-titer antinuclear antibodies, hypocomplementemia, lupus-specific autoantibodies, and mesangial proliferative lupus nephritis, confirming new-onset SLE. His management was complicated by latent tuberculosis, neutropenia, and cyclophosphamide-induced hearing loss. Despite intensive therapy with corticosteroids, rituximab, cyclophosphamide, obinutuzumab, plasmapheresis, and intravenous immunoglobulin, he remained profoundly insulin resistant, requiring very high daily insulin doses, with persistently elevated insulin receptor antibody titers. Initiation of daratumumab, a CD38 monoclonal antibody, resulted in marked clinical improvement, with progressive decline in insulin requirements and eventual discontinuation of insulin 17 months after presentation. He achieved durable remission with normalization of hemoglobin A1c. This unusually severe case of TBIRS highlights the limitations of conventional B-cell-directed therapies, where long-lived plasma cells sustain pathogenic autoantibodies. Plasma cell-targeted strategies such as daratumumab may represent an effective therapeutic option in refractory cases.
2026-03-13 | Clinical Characteristics and Risk Factors of Exogenous Insulin Antibody Syndrome in Patients with Diabetes: A Retrospective Cross-Sectional Study.
Insulin autoimmune syndrome (IAS) is a rare hypoglycemic disorder often confused with insulinoma or insulin overdose. Patients with diabetes on insulin therapy increasingly show insulin autoantibodies (IAAs), presenting symptoms similar to classic IAS, termed exogenous insulin antibody syndrome (EIAS). This study examines EIAS clinical features and risk factors. Patients with diabetes with IAA test results, admitted to our hospital between June 2023 and March 2024 were retrospectively enrolled. Participants were stratified into control and EIAS groups on the basis of IAA status. Clinical characteristics were compared between groups, independent risk factors for EIAS were identified by multivariate logistic regression, and the diagnostic utility of fasting insulin for predicting EIAS was assessed with receiver-operating-characteristic (ROC) curve analysis. Of 120 patients with diabetes and available IAAs results, 37 met criteria for EIAS. Compared with controls, EIAS patients were older, had longer diabetes duration, were more often treated with insulin aspart or premixed human insulin, and received higher daily insulin doses. Paradoxically, EIAS patients had markedly lower levels of fasting blood glucose and HbA1c, while higher fasting and 2-h post-prandial insulin concentrations, as well as HOMA-IR. Multivariate logistic regression analysis showed that elevated fasting insulin levels were independently associated with increased risk of EIAS. For every 1 uU/mL increase in fasting insulin, the risk of EIAS increased by 3% (OR = 1.03, 95% CI: 1.00-1.05). The fasting insulin level demonstrated high overall diagnostic and predictive efficacy for EIAS, with an area under the curve (AUC) of 0.782 (95% CI: 0.691-0.872). The optimal diagnostic cutoff value was 6.975 uU/mL, with a sensitivity of 73.0% and a specificity of 81.9%. EIAS patients were identified with advanced age, prolonged diabetes duration, high insulin dosage, hypoglycemia, and hyperinsulinemia. Fasting insulin level is independently associated with EIAS risk and demonstrates good diagnostic performance.
2026-02-05 | Three Interesting Cases of Hypoglycemia: A Case Series.
Hypoglycemia can be due to exogenous hyperinsulinemic hypoglycemia or endogenous hyperinsulinemic hypoglycemia (ENHH). ENHH is a rare entity characterized by inappropriately elevated plasma insulin in the background of low plasma glucose levels. ENHH, being an uncommon disease, is caused by many rare entities, with drug-induced being the most common cause of hypoglycemia. Other causes include insulinoma, insulin autoimmune syndrome, and nesidioblastosis. Here, we report three rare cases of non-diabetic hypoglycemia referred to the endocrinology clinic who had signs and symptoms of hypoglycemia fulfilling Whipple's triad with evidence of hyperinsulinemia despite low plasma glucose. All the appropriate biochemical investigations along with imaging studies were conducted to find out the etiology of hypoglycemia. After taking detailed medical history and clinical examination with biochemical and radiological investigations, patients were diagnosed with insulin autoimmune syndrome, insulinoma, and drug-induced hypoglycemia. Appropriate management was started, and patients were relieved of hypoglycemia. Diseases causing hypoglycemia have similar manifestation, but the diagnosis and management are always a challenging task. Hence, an accurate and early diagnosis is always a deciding factor for further prognosis.
2026-01-01 | Case Report on Hypoglycemia – Beyond Diet, Drugs and Diabetes
Hypoglycemia is a common presentation for various conditions presenting to the emergency department. After ruling out drugs as a cause, especially in nondiabetic individuals, classification to hyperinsulinemic or non-hyper insulinemic hypoglycemia is the next step. Since insulinoma is one of the most common causes of hyperinsulinemia, several imaging modalities are typically used in the search for it. However, in the presence of autoimmune autoantibody syndrome should be thought of. Here we report a case of an elderly non diabetic female with recurrent hypoglycemia. She was noticed to have late postprandial hypoglycemia. Investigation revealed raised insulin levels, raised C peptide levels, and high titers of insulin auto-antibodies. The patient was diagnosed with Insulin antibody syndrome (IAS). IAS may not be a rare syndrome; physicians need to be more vigilant to diagnose and manage such patients.
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Drug Discovery Landscape
1 orphan drug designation for Insulin autoimmune syndrome.
1 orphan drug designation for Insulin autoimmune syndrome.
Drug | Therapy type | Orphan designation | Clinical trial status | Sponsor |
|---|---|---|---|---|
Glucagon analogue linked to a human immunoglobulin Fc fragment | antibodies | EMA | — | JVM Europe B.V. |
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