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Type B insulin resistance syndrome in a patient with type 1 diabetes
Gävle Cent Hosp, Dept Internal Med, Div Endocrinol & Diabetol, Gävle, Sweden..ORCID iD: 0000-0002-5274-9748
Uppsala universitet, Klinisk diabetologi och metabolism.ORCID iD: 0000-0001-5498-3899
Uppsala universitet, Uppsala kliniska forskningscentrum (UCR).
Uppsala universitet, Njurmedicin.
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2020 (English)In: Endocrinology, diabetes & metabolism case reports, ISSN 2052-0573, Vol. 2020, no 1, article id 190157Article in journal (Refereed) Published
Abstract [en]

Type B insulin resistance syndrome (TBIRS) is a very rare autoimmune disorder with polyclonal autoantibodies against the insulin receptor, resulting in severe and refractory hyperglycemia. Described here is a patient who within a few months after the onset of autoimmune type 1 diabetes increased her insulin requirements more than 20-fold; despite this she had considerable difficulty maintaining a plasma glucose value of <40-60 mmol/L (720-1100 mg/dL). On suspicion of TBIRS, the patient was started on tapering dose of glucocorticoids to overcome the autoimmune insulin receptor blockade, resulting in an immediate and pronounced effect. Within days, insulin requirements decreased by 80-90% and plasma glucose stabilized around 7-8 mmol/L (126-144 mg/dL). The presence of antibodies to the insulin receptor was detected by immunoprecipitation and binding assays. After a 4-month remission on low maintenance dose prednisolone, the patient relapsed, which required repeated plasmaphereses and immune column treatments with temporarily remarkable effect. Mixed and transient results were seen with rituximab, mycophenolic acid and bortezomib, but the glycemic status remained suboptimal. Lack of compliance and recurrent infections may have contributed to this.

Place, publisher, year, edition, pages
Bioscientifica , 2020. Vol. 2020, no 1, article id 190157
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Endocrinology and Diabetes
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URN: urn:nbn:se:hig:diva-38217DOI: 10.1530/EDM-19-0157ISI: 000555811700001PubMedID: 32478674OAI: oai:DiVA.org:hig-38217DiVA, id: diva2:1646170
Available from: 2022-03-21 Created: 2022-03-21 Last updated: 2022-03-21Bibliographically approved

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Sjöholm, ÅkePereira, Maria JoãoNilsson, ThomasLinde, TorbjörnKatsogiannos, PetrosEriksson, Jan W.

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Sjöholm, ÅkePereira, Maria JoãoNilsson, ThomasLinde, TorbjörnKatsogiannos, PetrosEriksson, Jan W.
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