Kotani Masato, Tamura Naohisa, Inoue Tatsuhide, Tanaka Issei
Center for Diabetes, Endocrinology and Metabolism.
Research Support Center, Shizuoka General Hospital, Shizuoka, Shizuoka, Japan.
Endocrinol Diabetes Metab Case Rep. 2019 Nov 12;2019. doi: 10.1530/EDM-19-0115.
Type B insulin resistance syndrome is characterized by the presence of autoantibodies to the insulin receptor. We present a 57-year-old male admitted to a hospital due to body weight loss of 16 kg and hyperglycemia of 13.6 mmol/L. He was diagnosed with type B insulin resistance syndrome because the anti-insulin receptor antibodies were positive. We informed him that some hyperglycemic cases of this syndrome had been reported to be spontaneously remitted in 5 years, and he did not agree to be treated with high-dose glucocorticoids and/or immunosuppressive agents due to his concern for their adverse effects such as hyperglycemia and immunosuppression. He chose to be treated with insulin and voglibose, but fair glucose control could not be obtained. Six years later, he agreed to be treated with low-dose glucocorticoids practicable in outpatient settings. One milligram per day of betamethasone was tried orally and reduced gradually according to the values of glycated hemoglobin. After 30 months of glucocorticoid treatment, the anti-insulin receptor antibodies became undetectable and his fasting plasma glucose and glycated hemoglobin were normalized. This case suggests that low-dose glucocorticoids could be a choice to treat type B insulin resistance syndrome in outpatient settings.
Type B insulin resistance syndrome is an acquired autoimmune disease for insulin receptors. This case suggested the possibility of long-lasting, low-dose glucocorticoid therapy for the syndrome as an alternative for high-dose glucocorticoids or immunosuppressive agents. Since the prevalence of autoimmune nephritis is high in the syndrome, a delay of immunosuppressive therapy initiation might result in an exacerbation of nephropathy.
B型胰岛素抵抗综合征的特征是存在胰岛素受体自身抗体。我们报告一例57岁男性，因体重减轻16kg及血糖高达13.6mmol/L入院。因其抗胰岛素受体抗体呈阳性，故诊断为B型胰岛素抵抗综合征。我们告知他，该综合征的一些高血糖病例据报道在5年内可自发缓解，而他因担心高剂量糖皮质激素和/或免疫抑制剂的不良反应（如高血糖和免疫抑制），不同意接受此类治疗。他选择接受胰岛素和伏格列波糖治疗，但血糖控制不佳。6年后，他同意接受门诊可行的低剂量糖皮质激素治疗。尝试口服倍他米松每日1mg，并根据糖化血红蛋白值逐渐减量。糖皮质激素治疗30个月后，抗胰岛素受体抗体检测不到，空腹血糖和糖化血红蛋白恢复正常。该病例提示，低剂量糖皮质激素可作为门诊治疗B型胰岛素抵抗综合征的一种选择。
B型胰岛素抵抗综合征是一种获得性胰岛素受体自身免疫性疾病。该病例提示，对于该综合征，长期低剂量糖皮质激素治疗可能是高剂量糖皮质激素或免疫抑制剂治疗的替代方案。由于该综合征中自身免疫性肾炎的患病率较高，免疫抑制治疗启动延迟可能导致肾病加重。
