Division of Nephrology, Department of Medicine, Showa University School of Medicine, Tokyo, Japan.
Division of Diabetes, Metabolism and Endocrinology, Department of Medicine, Showa University School of Medicine, Tokyo, Japan.
Diabet Med. 2017 Dec;34(12):1788-1791. doi: 10.1111/dme.13524.
Type B insulin resistance syndrome is a rare disease characterized by refractory transient hyperglycaemia and severe insulin resistance associated with circulating anti-insulin receptor antibodies. A standardized treatment regimen for type B insulin resistance syndrome has yet to be established.
We report the case of a 64-year-old man undergoing haemodialysis for antineutrophil cytoplasmic antibody-associated vasculitis and diabetic nephropathy, who developed rapid onset of hyperglycaemia (glycated albumin 52.1%). Type B insulin resistance syndrome was diagnosed, on the basis of positivity for anti-insulin receptor antibodies and the man's autoimmune history of antineutrophil cytoplasmic antibody-associated vasculitis and idiopathic thrombocytopenic purpura. Although severe hyperglycaemia persisted in spite of corticosteroids and high-dose insulin therapy, rituximab treatment resulted in remarkable improvement of the man's severe insulin resistance and disappearance of anti-insulin receptor antibodies without any adverse effects.
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.
B 型胰岛素抵抗综合征是一种罕见疾病，其特征为难治性短暂性高血糖和严重胰岛素抵抗，与循环中的抗胰岛素受体抗体有关。目前尚未建立 B 型胰岛素抵抗综合征的标准化治疗方案。
我们报告了一例 64 岁男性病例，该患者因抗中性粒细胞胞质抗体相关性血管炎和糖尿病肾病而行血液透析，出现血糖迅速升高（糖化白蛋白 52.1%）。根据抗胰岛素受体抗体阳性和该患者的抗中性粒细胞胞质抗体相关性血管炎和特发性血小板减少性紫癜自身免疫史，诊断为 B 型胰岛素抵抗综合征。尽管给予皮质类固醇和大剂量胰岛素治疗，但严重高血糖仍持续存在，然而利妥昔单抗治疗使患者严重胰岛素抵抗显著改善，抗胰岛素受体抗体消失，且无任何不良反应。
根据文献复习的 11 例病例（包括本病例），利妥昔单抗似乎是治疗皮质类固醇抵抗型 B 型胰岛素抵抗综合征的一种安全有效的策略。
