Insulin Autoimmune Syndrome – A Case Series

Boro, Hiya; Gupta, Uttio; Singh, Charandeep; Malhotra, Rakhi; Khadgawat, Rajesh

doi:10.17925/ee.2020.16.2.168

Cited by 9 publications

(9 citation statements)

References 28 publications

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“…Therefore, it is particularly important to identify hypoglycemia caused by these two causes. Another differential diagnosis of IAS is hypoglycemia caused by advanced dumping syndrome [ 58 ].…”

Section: Resultsmentioning

confidence: 99%

Insulin Autoimmune Syndrome: A Systematic Review

Lin

Chen

Ning

2023

International Journal of Endocrinology

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Insulin autoimmune syndrome (IAS) is a rare endocrine disorder characterized by recurrent episodes of severe hypoglycemia, markedly elevated serum insulin, and positive insulin autoantibodies. In recent years, various countries have reported it one after another. It can be seen that we must pay attention to this disease. The diagnosis of IAS is challenging, requiring a careful workup aimed at excluding other causes of hyperinsulinemic hypoglycemia. High levels of insulin autoantibodies are found in patients, and C-peptide is not parallel to insulin, which could be diagnostic. IAS is a self-limiting disease with a good prognosis. Its treatment mainly includes symptomatic supportive treatment, such as adjusting the diet and using acarbose and other drugs to delay the absorption of glucose to prevent hypoglycemia. For patients with severe symptoms, available treatments may include drugs that reduce pancreatic insulin secretion (such as somatostatin and diazoxide), immunosuppressants (glucocorticoids, zaprin, and rituximab), and even plasma exchange to remove autoantibodies from the body. This review provides a comprehensive analysis of the epidemiology, pathogenesis, clinical manifestations, diagnosis and identification, and monitoring and treatment management of IAS.

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Section: Resultsmentioning

confidence: 99%

Insulin Autoimmune Syndrome: A Systematic Review

Lin

Chen

Ning

2023

International Journal of Endocrinology

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“…AIS seems to affect both genders equally [ 6 ], but this condition is rare among Indians, only 28 cases are reported in India. Usually reported in the seventh decade of life, however, nowadays age at onset varies [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

An Unusual Case of Hypoglycemia in a Non-diabetic Individual due to Hirata Disease

Padarabinda Tripathy,

Pathi,

Kumar Behera

et al. 2024

Cureus

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Hypoglycemia is common in diabetic populations using insulin or insulin secretagogues, but rare in non-diabetics. A 60-year-old non-diabetic male presented with repeated episodes of abnormal behavior persisting for 10-15 minutes for seven days, associated with sweating, intense hunger, and relief on food intake, with no history of insulin or secretagogue intake, with stable vitals and normal systemic examination. Laboratory tests during attacks revealed low blood sugar, high serum insulin, and normal C-peptide levels, with no evidence of pancreatic or extrapancreatic hyperinsulinism, and serum anti-insulin antibody levels >100 U/ml. Based on these results, he was diagnosed with autoimmune insulin syndrome (AIS). Treatment with low-carb meals, oral prednisolone, and acarbose led to the resolution of symptoms. Hirata syndrome, though rare in India, requires consideration as a differential diagnosis to avoid unnecessary invasive procedures.

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“…This syndrome presents commonly in adults. Based on the available information, this condition is predominantly prevalent in Japanese populations, and up until the year 2020, there had been merely 28 documented cases reported in India [ 2 ]. The exact prevalence is disputed as it has probably been underestimated due to the lack of awareness of the disease, and difficulties in diagnostic workup, particularly in rural areas.…”

Section: Discussionmentioning

confidence: 99%

“…The presence of human leukocyte antigen (HLA)-DR4 and HLA-DRB1*0406 in East Asian patients and HLA-DRB1*0403 in Caucasian patients has been observed in IAS [ 2 ]. The mechanism of hypoglycemia in IAS is assumed to be due to high levels of IAA and is commonly associated with other autoimmune diseases such as Graves' disease, systemic lupus erythematosus, rheumatoid arthritis, and ankylosing spondylitis [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

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A Case of Insulin Autoimmune Syndrome in an Indian Male Taking Alpha-Lipoic Acid

Sehgal,

Ohri,

Mittal

et al. 2023

Cureus

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Insulin autoimmune syndrome [IAS, Hirata disease (HD)] is a rare cause of recurrent spontaneous hypoglycemic episodes, characterized by high serum insulin levels and high titers of autoantibodies against endogenous insulin. We report a case of a previously healthy Indian male presenting with recurrent episodes of hypoglycemia with no prior exposure to exogenous insulin. Regular glucose monitoring was done. Laboratory tests showed insulin >1000 μIU/mL and C-peptide levels of 12.8 ng/ml. The patient had high titers of insulin autoantibodies (IAA) (>100 units/mL; normal range: <10 units/mL), which indicated a diagnosis of IAS. The patient was consuming alpha-lipoic acid; sulfhydryl-containing compounds have been linked to IAS. This case report highlights the importance of IAA titers in first-line investigations for hypoglycemia in a non-diabetic patient with strikingly high blood insulin levels and discusses the potential relationship between IAS and alpha-lipoic acid.

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Insulin Autoimmune Syndrome – A Case Series

Cited by 9 publications

References 28 publications

Insulin Autoimmune Syndrome: A Systematic Review

Insulin Autoimmune Syndrome: A Systematic Review

An Unusual Case of Hypoglycemia in a Non-diabetic Individual due to Hirata Disease

A Case of Insulin Autoimmune Syndrome in an Indian Male Taking Alpha-Lipoic Acid

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