Abstract：
BACKGROUND: Type B insulin resistance syndrome is a rare form of diabetes due to the presence of anti-insulin receptor antibodies [1, 2], which causes glycemic decompensation and antidiabetic therapy failure and instead responds to immunosuppressive therapy.
METHODS: A 67-year-old patient was admitted to the hospital due to autoimmune hemolytic anemia and glycemic decompensation. We first prescribed subcutaneous basal-bolus insulin and then intravenous insulin without improvement in blood sugar levels (between 300 and 500 mg/dL). Considering the non-response to therapy and the autoimmune diathesis of the patient (hemolytic anemia and mixed connective tissue disease), we suspected an autoimmune etiopathogenesis of glycemic decompensation; we excluded type 1 diabetes mellitus (specific antibodies were negative), and we considered the anti-insulin-antibodies-(-assayed and negative) and anti-insulin receptor antibodies (not assayed due to the lack of a center specialized in this assay in the area). Therefore, we decided to start Rituximab. After 2 weeks from the infusion, the patient improved glycemic compensation, reducing insulin requirement. Further, 2 months after the first infusion, the patient stopped insulin, returning to oral therapy with Metformin. To date, the patient has completed 3 cycles of Rituximab with the benefit of glycemic control (HbA1c 6.7%).
CONCLUSIONS: The brilliant response to Rituximab supports the hypothesis of an autoimmune pathogenesis. The anti-insulin receptor antibodies (in the type B insulin resistance syndrome) affect mostly middle-aged adults, especially women, in the context of other autoimmune diseases. Hence, it is necessary to consider the diagnosis of this rare disease in order to perform timely and effective treatment.
METHODS: A 67-year-old patient was admitted to the hospital due to autoimmune hemolytic anemia and glycemic decompensation. We first prescribed subcutaneous basal-bolus insulin and then intravenous insulin without improvement in blood sugar levels (between 300 and 500 mg/dL). Considering the non-response to therapy and the autoimmune diathesis of the patient (hemolytic anemia and mixed connective tissue disease), we suspected an autoimmune etiopathogenesis of glycemic decompensation; we excluded type 1 diabetes mellitus (specific antibodies were negative), and we considered the anti-insulin-antibodies-(-assayed and negative) and anti-insulin receptor antibodies (not assayed due to the lack of a center specialized in this assay in the area). Therefore, we decided to start Rituximab. After 2 weeks from the infusion, the patient improved glycemic compensation, reducing insulin requirement. Further, 2 months after the first infusion, the patient stopped insulin, returning to oral therapy with Metformin. To date, the patient has completed 3 cycles of Rituximab with the benefit of glycemic control (HbA1c 6.7%).
CONCLUSIONS: The brilliant response to Rituximab supports the hypothesis of an autoimmune pathogenesis. The anti-insulin receptor antibodies (in the type B insulin resistance syndrome) affect mostly middle-aged adults, especially women, in the context of other autoimmune diseases. Hence, it is necessary to consider the diagnosis of this rare disease in order to perform timely and effective treatment.
摘要：
背景：B型胰岛素抵抗综合征是一种罕见的糖尿病形式，由于存在抗胰岛素受体抗体[1，2]，导致血糖代偿失调和抗糖尿病治疗失败，而对免疫抑制治疗有反应。
方法：一名67岁患者因自身免疫性溶血性贫血和血糖代偿失调入院。我们首先规定皮下推注胰岛素，然后静脉内胰岛素，而血糖水平没有改善（300至500mg/dL）。考虑到患者对治疗无反应和自身免疫素质（溶血性贫血和混合性结缔组织病），我们怀疑血糖代偿失调的自身免疫性病因;我们排除了1型糖尿病（特异性抗体为阴性），我们考虑了抗胰岛素抗体（-测定和阴性）和抗胰岛素受体抗体（由于该领域缺乏专门研究该测定的中心而未进行测定）。因此,我们决定开始使用利妥昔单抗.从输液2周后，患者改善了血糖补偿，减少胰岛素需求。Further,第一次输液后2个月，病人停止了胰岛素，回到二甲双胍口服治疗。迄今为止,患者已完成3个周期的利妥昔单抗治疗,且获益于血糖控制(HbA1c6.7%).
结论：对利妥昔单抗的明亮反应支持自身免疫发病机制的假说。抗胰岛素受体抗体（B型胰岛素抵抗综合征）主要影响中年人，尤其是女性,在其他自身免疫性疾病的背景下。因此，有必要考虑这种罕见疾病的诊断，以便进行及时有效的治疗。
方法：一名67岁患者因自身免疫性溶血性贫血和血糖代偿失调入院。我们首先规定皮下推注胰岛素，然后静脉内胰岛素，而血糖水平没有改善（300至500mg/dL）。考虑到患者对治疗无反应和自身免疫素质（溶血性贫血和混合性结缔组织病），我们怀疑血糖代偿失调的自身免疫性病因;我们排除了1型糖尿病（特异性抗体为阴性），我们考虑了抗胰岛素抗体（-测定和阴性）和抗胰岛素受体抗体（由于该领域缺乏专门研究该测定的中心而未进行测定）。因此,我们决定开始使用利妥昔单抗.从输液2周后，患者改善了血糖补偿，减少胰岛素需求。Further,第一次输液后2个月，病人停止了胰岛素，回到二甲双胍口服治疗。迄今为止,患者已完成3个周期的利妥昔单抗治疗,且获益于血糖控制(HbA1c6.7%).
结论：对利妥昔单抗的明亮反应支持自身免疫发病机制的假说。抗胰岛素受体抗体（B型胰岛素抵抗综合征）主要影响中年人，尤其是女性,在其他自身免疫性疾病的背景下。因此，有必要考虑这种罕见疾病的诊断，以便进行及时有效的治疗。
