Edem Dinesh, Maradana Jhansi, Majety Priyanka, Antony Mc Anto, Menon Lakshmi
Endocrinology, Diabetes and Metabolism, University of Arkansas for Medical Sciences, Little Rock, USA.
Endocrinology, Diabetes and Metabolism, Mass General Brigham Wentworth-Douglas Hospital, Dover, USA.
Cureus. 2024 Apr 14;16(4):e58270. doi: 10.7759/cureus.58270. eCollection 2024 Apr.
Insulin autoimmune syndrome (IAS) or Hirata disease is a rare condition presenting as recurrent hypoglycemia, and associated with elevated insulin levels in the presence of insulin autoantibodies (IAAs) in patients who were never exposed to exogenous insulin and with no evidence of pancreatic abnormalities. IAS is much more frequent in East Asians, especially the Japanese population, compared to the lower incidence in Caucasians. However, it can be associated with other autoimmune diseases or drug use like methimazole and alpha-lipoic acid (ALA). We report a case of a 47-year-old Caucasian male presenting with a 12-month history of worsening episodes of fasting and post-prandial hypoglycemia associated with symptoms of dizziness, tremors, palpitations, and unconsciousness associated with hypoglycemia. Symptoms resolved with the administration of carbohydrate-containing foods, establishing Whipple's triad. At an outside facility, he had initial labs that showed elevated insulin levels (141 µU/ml) with normal glucose, C-peptide, and proinsulin levels, but there was no availability of an IAA lab assay. Given his symptoms, severity, and frequency of hypoglycemia, he was admitted to the hospital for a 72-hour fast, which showed the lowest glucose level of 64 mg/dl with inappropriately high insulin of 22.2 µU/ml, low C-peptide of 0.57 ng/ml, and undetectable proinsulin of <1.6 pmol/L, but with IAA being >50 U/ml (0.0-0.4 U/ml). He was treated with intensive dietary counseling with a low-carbohydrate diet and prednisone 20 mg twice daily initially. Additionally, he could not tolerate octreotide, diazoxide, and acarbose due to side effects. He is currently on prednisone 10 mg daily and nifedipine with no further hypoglycemic episodes, but still has a high IAA of >50 U/ml and serum insulin levels of 70-112 µU/ml. Our case highlights the importance of recognizing hypoglycemia and checking for IAA levels as first-line diagnostic tests, in the absence of which there could be a delay in diagnosis and leading to unnecessary lab and imaging testing. Our case is unique since it happened in a Caucasian without any prior exposure to a triggering factor and has not undergone self-remission yet, which happens in most of IAS cases.
胰岛素自身免疫综合征（IAS）或平田病是一种罕见疾病，表现为反复低血糖，在从未接触过外源性胰岛素且无胰腺异常证据的患者中，存在胰岛素自身抗体（IAA）时胰岛素水平升高。与白种人较低的发病率相比，IAS在东亚人中更为常见，尤其是日本人群。然而，它可能与其他自身免疫性疾病或药物使用有关，如甲巯咪唑和α-硫辛酸（ALA）。我们报告一例47岁白种男性病例，该患者有12个月的空腹和餐后低血糖发作加重病史，伴有头晕、震颤、心悸等症状以及与低血糖相关的意识丧失。给予含碳水化合物食物后症状缓解，确立了惠普尔三联征。在外部机构，他最初的实验室检查显示胰岛素水平升高（141 μU/ml），而血糖、C肽和胰岛素原水平正常，但无法进行IAA实验室检测。鉴于他的症状、低血糖的严重程度和发作频率，他被收住入院进行72小时禁食试验，结果显示最低血糖水平为64 mg/dl，胰岛素水平不适当升高至22.2 μU/ml，C肽水平低至0.57 ng/ml，胰岛素原水平检测不到<1.6 pmol/L，但IAA>50 U/ml（0.0 - 0.4 U/ml）。最初对他进行了强化饮食咨询，采用低碳水化合物饮食，并给予泼尼松20 mg每日两次。此外，由于副作用，他无法耐受奥曲肽、二氮嗪和阿卡波糖。他目前每天服用10 mg泼尼松和硝苯地平，未再发生低血糖发作，但IAA仍>50 U/ml，血清胰岛素水平为70 - 112 μU/ml。我们的病例强调了在没有低血糖发作时识别低血糖并检查IAA水平作为一线诊断测试的重要性，否则可能会延迟诊断并导致不必要的实验室和影像学检查。我们的病例很独特，因为它发生在一名白种人身上，之前没有接触过任何触发因素，且尚未自行缓解，而大多数IAS病例会自行缓解。
