Division of Endocrinology, Metabolism, and Diabetes (D.R.S., M.T.M.), University of Colorado, Anschutz Medical Campus, Aurora, Colorado 80045; Research Service (D.R.S.), Denver Veterans Affairs Medical Center, Denver, Colorado 80220; and Barbara Davis Center for Childhood Diabetes (A.W.M.), Anschutz Medical Campus, Aurora, Colorado 80045.
J Clin Endocrinol Metab. 2016 May;101(5):1931-4. doi: 10.1210/jc.2016-1097. Epub 2016 Mar 16.
Insulin autoimmune syndrome (IAS), or Hirata's disease, is a rare hypoglycemic disorder characterized by spontaneous hypoglycemia without evidence of exogenous insulin administration, a high serum concentration of total immunoreactive insulin, and the presence of insulin autoantibodies in high titer. The majority of cases occur in the Asian population, and treatment is generally successful with watchful waiting or steroids.
We report the case of a 71-year-old Caucasian man with severe hypoglycemia due to IAS that was refractory to a prolonged course of high-dose steroids. Type 1 diabetes prevention trials using rituximab have shown selective suppression of insulin autoantibodies, which are the pathogenic antibodies in IAS, and therefore we provided this therapy. Treatment with two doses of rituximab and daily use of a continuous glucose monitor resulted in disease remission.
We present the first case where potentially life-threatening hypoglycemia due to IAS was successfully managed with rituximab and a continuous glucose monitor. We conclude that these treatment modalities are effective for the management of severe, refractory IAS.
胰岛素自身免疫综合征（IAS），或平田病，是一种罕见的低血糖疾病，其特征为自发性低血糖，无外源性胰岛素给药的证据，总免疫反应性胰岛素血清浓度高，以及存在高滴度的胰岛素自身抗体。大多数病例发生在亚洲人群中，一般通过密切观察或类固醇治疗即可成功治愈。
我们报告了一例 71 岁的高加索男性病例，因 IAS 导致严重低血糖，且对长期大剂量类固醇治疗无效。使用利妥昔单抗的 1 型糖尿病预防试验表明，该疗法可选择性抑制 IAS 的致病抗体——胰岛素自身抗体，因此我们采用了该疗法。两剂利妥昔单抗治疗和每日使用连续血糖监测使疾病得到缓解。
我们报告了首例因 IAS 导致的潜在危及生命的低血糖症，通过利妥昔单抗和连续血糖监测成功得到了控制。我们得出结论，这些治疗方法对于严重、难治性 IAS 的治疗是有效的。
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