Rituximab for the treatment of type B insulin resistance syndrome: a case report and review of the literature

Iseri, Ken; Iyoda, Masayuki; Shikida, Yasuto; Inokuchi, Takako; Morikawa, Tomoki; Hara, Naoyuki; Hirano, Tsutomu; Shibata, Takanori

doi:10.1111/dme.13524

Cited by 14 publications

(12 citation statements)

References 11 publications

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“…Rituximab, an anti-CD20 monoclonal antibody, proved particularly efficient in reducing anti-insulin receptor autoantibodies. Since its use was first described by Coll et al (123), several cases of TBIRS have reportedly been successfully treated with rituximab (124). Very often, these agents were used in combination, however, making it very difficult to establish which therapeutic agent induced the remission.…”

Section: Treatmentmentioning

confidence: 99%

Insulin autoimmune syndrome: from diagnosis to clinical management

2018

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Autoimmune forms of hypoglycemia are a rare cause of low blood sugar levels among Caucasians, and often go misdiagnosed, exposing patients to lengthy series of pointless, potentially harmful and expensive tests. There are two types of autoimmune hypoglycemia. One is insulin autoimmune syndrome (IAS), which is characterized by hyperinsulinemic hypoglycemia, elevated insulin autoantibody (IAA) titers, no prior exposure to exogenous insulin, and no of pathological abnormalities of the pancreatic islets. This condition is also known as "Hirata's disease". The other is type B insulin resistance syndrome (TBIRS), a rare autoimmune disorder resulting in a broad array of abnormalities in glucose homeostasisfrom hypoglycemia to extremely insulin-resistant hyperglycemia-caused by the presence of insulin receptor autoantibodies (IRAbs). This review focuses on these two syndromes, describing their epidemiology, possible genetic background, clinical presentation, pathophysiology, diagnosis and treatment.

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Section: Treatmentmentioning

confidence: 99%

Insulin autoimmune syndrome: from diagnosis to clinical management

2018

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“…Rituximab, an anti-CD20 monoclonal antibody, depletes CD20+ B cells and reconstitutes the peripheral B cell lineage (13). Rituximab in combination with cyclophosphamide and a high-dose pulsed corticosteroid induced remission in insulin-refractory patients, as did a combination of intravenous immunoglobulin and plasmapheresis (14), and corticosteroid therapy (15). Recently, complete remission of type B insulin-resistance after immunotherapy that included rituximab has been described in several case reports (16)(17)(18).…”

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confidence: 99%

Paradigm shift in the management of type B insulin resistance

et al. 2018

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“…We used the protocol established by Klubo-Gwiezdzinska et al ( 8 ), using immunosuppressive therapy based on cycles of methylprednisolone 1 g IV for 2 days and cyclophosphamide 50 mg (one tablet) BID. Rituximab, a monoclonal anti-CD20 antibody, was included in this protocol, since it previously showed high efficacy and safety in inducing initial remission ( 1 , 7 , 13 ); however, it was not administered in the present case since it is not covered for this disease by Peruvian health insurance and patients cannot obtain it due to its high cost, restricting its use significantly. This protocol led to a significant reduction in previously reported mortality rates ( 14 ).…”

Section: Discussionmentioning

confidence: 99%

Successful remission of type B insulin resistance syndrome without rituximab in an elderly male

Concepción-Zavaleta

Ildefonso-Najarro

Dueñas

et al. 2020

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary Type B insulin resistance syndrome (TBIR) is a rare autoimmune disease caused by antibodies against the insulin receptor. It should be considered in patients with dysglycaemia and severe insulin resistance when other more common causes have been ruled out. We report a case of a 72-year-old male with a 4-year history of type 2 diabetes who presented with hypercatabolism, vitiligo, acanthosis nigricans, and hyperglycaemia resistant to massive doses of insulin (up to 1000 U/day). Detection of anti-insulin receptor antibodies confirmed TBIR. The patient received six pulses of methylprednisolone and daily treatment with cyclophosphamide for 6 months. Response to treatment was evident after the fourth pulse of methylprednisolone, as indicated by weight gain, decreased glycosylated haemoglobin and decreased requirement of exogenous insulin that was later discontinued due to episodes of hypoglycaemia. Remission was eventually achieved and the patient is currently asymptomatic, does not require insulin therapy, has normal glycaemia and is awaiting initiation of maintenance therapy with azathioprine. Thus, TBIR remitted without the use of rituximab. This case highlights the importance of diagnosis and treatment in a timely fashion, as well as the significance of clinical features, available laboratory findings and medication. Large controlled studies are required to standardise a therapeutic protocol, particularly in resource-constrained settings where access to rituximab is limited. Learning points: Type B insulin resistance syndrome is a rare autoimmune disorder that should be considered in patients with dysglycaemia, severe insulin resistance and a concomitant autoimmune disease. Serological confirmation of antibodies against the insulin receptor is not necessary in all cases due to the high associated mortality without timely treatment. Although there is no standardised immunosuppressive treatment, a protocol containing rituximab, cyclophosphamide and steroids has shown a significant reduction in previously reported mortality rates. The present case, reports successful remission in an atypical patient using cyclophosphamide and methylprednisolone, which is an effective therapy in countries in which rituximab is not covered by health insurance. When there is improvement in the hypercatabolic phase, the insulin dose should be reduced and/or discontinued to prevent hypoglycaemia; a mild postprandial hyperglycaemic state should be acceptable.

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Rituximab for the treatment of type B insulin resistance syndrome: a case report and review of the literature

Abstract: According to a literature review of 11 cases in addition to the present case, rituximab appears to be a safe and effective strategy for the treatment of corticosteroid-resistant type B insulin resistance syndrome.

Cited by 14 publications

References 11 publications

Insulin autoimmune syndrome: from diagnosis to clinical management

Insulin autoimmune syndrome: from diagnosis to clinical management

Paradigm shift in the management of type B insulin resistance

Successful remission of type B insulin resistance syndrome without rituximab in an elderly male

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