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      Type B insulin resistance syndrome: a systematic review

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          ABSTRACT

          A literature review on the clinical, laboratory, and treatment features of type B insulin resistance syndrome (TBIRS). Data from PubMed, the Virtual Health Library and Cochrane database were selected and analyzed using the REDCap application and R statistical program. From 182 papers, 65 were selected, which assessed 119 clinical cases, 76.5% in females and 42.9% in African-Americans, with an average age of 44 years. A common feature of TBIRS is co-occurrence of autoimmune diseases, such as systemic lupus erythematosus (most frequently reported). Hyperglycemia of difficult control was the mostly reported condition. Tests for anti-insulin receptor antibodies were positive in 44.2% of the cases. Disease management comprised fractional diet, insulin therapy (maximum dose given was 57 600 IU/day), plasmapheresis and immunosuppression with several classes of drugs, mainly glucocorticoids. Remission occurred in 69.7% of cases, in 30.3% of these spontaneously. The mortality rate was 15.38%. There was an inverse relationship between anti-insulin antibodies and remission (p = 0.033); and a positive correlation between combined immunosuppressive therapy and remission (p = 0.002). Relapse occurred in 7.6% of the cases. This rare syndrome has difficult-to-control diabetes, even with high doses of insulin, and it is usually associated with autoimmune diseases. Therapeutic advances using immunomodulatory therapy have led to significant improvements in the rate of remission.

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          Most cited references69

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          Insulin resistance--mechanisms, syndromes, and implications.

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            Clinical course of the syndrome of autoantibodies to the insulin receptor (type B insulin resistance): a 28-year perspective.

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              Treatment of type B insulin resistance: a novel approach to reduce insulin receptor autoantibodies.

              Type B insulin resistance belongs to a class of diseases caused by an autoantibody to a cell surface receptor. Blockade of insulin action results in hyperglycemia, hypercatabolism, severe acanthosis nigricans, and hyperandrogenism in women. This rare autoimmune disorder has been treated with various forms of immunosuppression with mixed success. We describe 14 patients with type B insulin resistance referred to the National Institutes of Health, adding to an existing cohort of 24 patients. This report focuses on seven patients who were treated with an intensive combination protocol of rituximab, cyclophosphamide, and pulse corticosteroids aimed at control of pathogenic autoantibody production. Hematological, metabolic, and endocrine parameters, including fasting glucose, glycated hemoglobin, insulin dose, lipids, and testosterone, were monitored before and after treatment. All seven treated patients achieved remission, defined as amelioration of hyperglycemia, discontinuation of insulin therapy, and resolution of hyperandrogenism. Glycated hemoglobin has normalized in all seven treated patients. Remission was achieved on average in 8 months from initiation of treatment. The medication regimen was well tolerated, with no serious adverse events. In seven patients with type B insulin resistance, standardized treatment with rituximab, cyclophosphamide, and pulse steroids results in remission of the disease. Future studies will determine whether this treatment protocol can be applied to other autoantibody/cell surface receptor disease states.
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                Author and article information

                Journal
                Arch Endocrinol Metab
                Arch Endocrinol Metab
                aem
                Archives of Endocrinology and Metabolism
                Sociedade Brasileira de Endocrinologia e Metabologia
                2359-3997
                2359-4292
                27 May 2020
                Jul-Aug 2020
                : 64
                : 4
                : 337-348
                Affiliations
                [1 ] orgdiv2Hospital Universitário Walter Cantídio orgdiv1Faculdade de Medicina orgnameUniversidade Federal do Ceará Fortaleza CE Brasil originalHospital Universitário Walter Cantídio, Faculdade de Medicina, Universidade Federal do Ceará (UFC), Fortaleza, CE, Brasil
                Author notes
                Correspondence to: Renan Magalhães Montenegro Junior Hospital Universitário Walter Cantídio, Faculdade de Medicina, Universidade Federal do Ceará (UFC), Fortaleza, CE, Brasil renanmmjr@ 123456gmail.com

                Disclosure: no potential conflict of interest relevant to this article was reported.

                Author information
                https://orcid.org/0000-0002-2196-9326
                https://orcid.org/0000-0002-4311-6551
                https://orcid.org/0000-0001-9329-5343
                https://orcid.org/0000-0002-7458-7997
                https://orcid.org/0000-0001-6198-3467
                https://orcid.org/0000-0001-7287-8726
                Article
                2359-3997000000257
                10.20945/2359-3997000000257
                10522085
                32813762
                ef9c0203-869d-4be7-babe-ad6fb2f8d173

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 3 June 2019
                : 3 February 2020
                Page count
                Figures: 3, Tables: 2, Equations: 0, References: 65
                Categories
                Review

                anti-insulin receptor antibody,insulin resistance syndrome,diabetes, autoimmunity

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