Department of Endocrinology, Waikato District Health Board, Hamilton, New Zealand
Department of Biochemistry, Waikato District Health Board, Hamilton, New Zealand.
BMJ Case Rep. 2022 Feb 19;15(2):e246523. doi: 10.1136/bcr-2021-246523.
Type B insulin resistance syndrome is a rare autoimmune disorder affecting glucose homeostasis characterised by the presence of serum autoantibodies to the insulin receptor. Typically, these patients present with severe insulin resistance although a mixed hyperglycaemic and hypoglycaemic phenotype may also occur, as can an exceptionally rare isolated hypoglycaemia presentation. The classic biochemical pattern comprises elevated insulin levels despite significant hypoglycaemia. We report an adult man presenting with isolated hypoglycaemia and suppressed serum insulin and C-peptide levels. He demonstrated evidence of autoimmunity with positive antinuclear antibodies, reactive lymphadenopathy and cytopaenias but did not meet the criteria for systemic lupus erythematosus and underlying malignancy was not identified despite extensive investigation. Insulin receptor antibodies were present. Treatment with prednisone led to resolution of hypoglycaemia, with no recurrence after 36 months of follow-up. However, 42 months after initial presentation, he represented with high-grade lymphoma.
B 型胰岛素抵抗综合征是一种罕见的自身免疫性疾病，影响葡萄糖稳态，其特征是存在针对胰岛素受体的血清自身抗体。这些患者通常表现为严重的胰岛素抵抗，尽管也可能出现混合性高血糖和低血糖表型，也可能出现极其罕见的孤立性低血糖表现。典型的生化模式包括尽管存在严重低血糖但胰岛素水平升高。我们报告了一名成年男性，表现为孤立性低血糖和血清胰岛素和 C 肽水平抑制。他表现出自身免疫的证据，包括抗核抗体阳性、反应性淋巴结病和细胞减少症，但不符合系统性红斑狼疮的标准，尽管进行了广泛的检查，但未发现潜在恶性肿瘤。存在胰岛素受体抗体。泼尼松治疗导致低血糖缓解，随访 36 个月后无复发。然而，在初次就诊后 42 个月，他出现了高级别淋巴瘤。
