Intractable Hypoglycemia in the Setting of Autoimmune Overlap Syndrome

Shanker, Kanika; Daley, Tanicia; Semple, Robert K.; Rouster‐Stevens, Kelly; Ham, J. Nina

doi:10.1542/peds.2016-0866

Cited by 7 publications

(9 citation statements)

References 12 publications

(17 reference statements)

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“…Patient number 1 had an unusual presentation which is consistent with what has been listed as autoimmune overlap in western literature. [15] While steroids caused remission of the IAAS she went on to develop another autoimmune condition which required use of plasmapheresis.…”

Section: Discussionmentioning

confidence: 99%

Insulin autoantibody syndrome: Varying clinical presentations and response patterns of an underrecognized entity

Manjunath

Pavithran

Bhavani

et al. 2019

Indian J Endocr Metab

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Context:Insulin autoantibody syndrome (IAAS) is considered to be a rare cause of hyperinsulinaemic hypoglycaemia. Lack of familiarity with the varied clinical manifestations leads to underdiagnosis. Localization techniques aimed at insulin-secreting neoplasms and nesidioblastosis, which are expensive often are ordered when the correct diagnosis is not made.Aims:We describe the myriad of clinical manifestations associated with IAAS based on single centre experience.Settings and Design:Retrospective analysis of patients who got admitted with symptoms suggestive of hypoglycaemia and underwent mixed meal test and prolonged hypoglycaemic test from 2016 to 2019.Subjects and Methods:Retrospective data of 12 patients with IAAS who were diagnosed in the threeyear time period between 2016 and 2019 are included in this analysis. Clinical details, biochemical parameters and imaging modalities were analysed.Statistical Analysis:All analyses were performed with SPSS software (version 17).Results:Total of twelve patients 12 (5 male and 7 females) were identified as IAAS. Median age of presentation was 57 years. Median insulin levels and median C-peptide levels were 300 miu/ml and 18.5 ng/ml respectively. Only 3 (25%) patients had spontaneous resolution. Steroid induced remission occurred by 3 months in the remaining patients. Intermittent hyperglycaemia was seen in 9 (75%) patients. Implicatable drug use preceding the occurrence of the clinical symptoms was observed in five patients.Conclusion:IAAS is not uncommon in India. The diagnosis should be pursued in patients with hyperinsulinaemic hypoglycaemia especially when insulin levels are very high or when there is intermittent hyperglycaemia.

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Section: Discussionmentioning

confidence: 99%

Insulin autoantibody syndrome: Varying clinical presentations and response patterns of an underrecognized entity

Manjunath

Pavithran

Bhavani

et al. 2019

Indian J Endocr Metab

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“…Women often have enlarged ovaries and elevated testosterone levels. (17,18,19,20,21); besides, some patients are treated with plasmapheresis (17,18,19)); however, a remission can be achieved only in 50% of cases (17). Mortality rates are high (18): up to 54% of patients die within 10 years after diagnosis (21).…”

Section: Introductionmentioning

confidence: 99%

“…Patients with this syndrome are usually erroneously diagnosed with type 2 diabetes mellitus ( 18 ), less frequently with type 1 diabetes ( 19 ), as hyperglycaemia is often observed at the onset of the disease. Acanthosis nigricans in the axillary and inguinal regions, the neck, around the eyes ( 17 ), and around the mouth ( 14 ) is a typical, frequently seen feature of this condition.…”

Section: Introductionmentioning

confidence: 99%

“…According to Arioglu et al ., one-third of patients with type B insulin resistance develop a spontaneous remission within 11–48 months. Otherwise, the patient requires pharmacological therapy (hypoglycaemia should be treated with glucocorticoids, cyclosporin A, azathioprine, cyclophosphamide, mycophenolate mofetil, and rituximab ( 17 , 18 , 19 , 20 , 21 ); besides, some patients are treated with plasmapheresis ( 17 , 18 , 19 )); however, a remission can be achieved only in 50% of cases ( 17 ). Mortality rates are high ( 18 ): up to 54% of patients die within 10 years after diagnosis ( 21 ).…”

Section: Introductionmentioning

confidence: 99%

“…Mortality rates are high ( 18 ): up to 54% of patients die within 10 years after diagnosis ( 21 ). The unfavourable prognosis is both due to the underlying condition and severe hypoglycaemic episodes ( 19 ).…”

Section: Introductionmentioning

confidence: 99%

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Insulin autoimmune syndrome

Yukina

Nuralieva

Solovyev

et al. 2020

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary Insulin autoimmune syndrome (Hirata’s disease) is a disorder caused by development of autoantibodies to insulin and manifested by hypoglycaemic syndrome. The overwhelming majority of physicians do not include it in the differential diagnosis of hypoglycaemic states because of a misconception of an extremely low prevalence of this condition. This results in unnecessary drug therapy and unjustified surgical interventions in patients that otherwise would be successfully treated conservatively. This disease is strongly associated with certain alleles of the HLA gene. In most cases, this condition develops in predisposed individuals taking drugs containing sulfhydryl groups. Formation of autoantibodies to insulin may be observed in patients with other autoimmune disorders, as well as in those with multiple myeloma or monoclonal gammopathy of undetermined significance. This paper presents the first Russian case report of insulin autoimmune syndrome in an adult patient. Learning points: Insulin autoimmune syndrome, Hirata’s disease, anti-insulin antibodies, and hypoglycaemia.

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Autoimmune Hypoglycaemia

Görden¹,

Malandrino²

2022

Oxford Textbook of Endocrinology and Diabetes 3e

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Insulin autoimmune syndrome and type B insulin resistance are rare causes of autoimmune hypoglycaemia. Insulin autoimmune syndrome is due to autoantibodies against endogenous insulin, and typically presents with episodes of postprandial hyperinsulinaemic hypoglycaemia. Type B insulin resistance is caused by autoantibodies against the insulin receptor, and may present with severe insulin resistance and hyperglycaemia, hypoglycaemia following a period of hyperglycaemia, or hypoglycaemia alone. The combination of clinical manifestations and positive insulin or insulin-receptor antibodies in the blood confirm the diagnosis of autoimmune hypoglycaemia. However, in rare cases, insulin treatment in patients with positive insulin antibodies and frequent episodes of hypoglycaemia can complicate the diagnosis of insulin autoimmune syndrome. In this chapter, the clinical characteristics, pathophysiology, diagnosis, and management of autoimmune hypoglycaemia syndromes will be described, and the challenges in identifying those insulin antibodies which may significantly affect insulin kinetics in insulin-treated diabetes discussed.

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Intractable Hypoglycemia in the Setting of Autoimmune Overlap Syndrome

Cited by 7 publications

References 12 publications

Insulin autoantibody syndrome: Varying clinical presentations and response patterns of an underrecognized entity

Insulin autoantibody syndrome: Varying clinical presentations and response patterns of an underrecognized entity

Insulin autoimmune syndrome

Autoimmune Hypoglycaemia

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