Heydari Iraj, Maghsoomi Zohreh, Hatami Neda, Soltani Pedram
Endocrine Research Center, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), Tehran, Iran.
Research Center for Prevention of Cardiovascular Disease, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), No. 10, Firoozeh St, Vali-asr Ave, Vali-asr Sq, 1593716615, Tehran, Iran.
J Med Case Rep. 2025 May 2;19(1):204. doi: 10.1186/s13256-025-05219-w.
Hirata syndrome is a rare cause of hypoglycemia characterized by high insulin levels and the presence of insulin autoantibodies. This case report aims to introduce Hirata syndrome as a cause of fasting hypoglycemia in a patient with autoimmune diseases, such as vitiligo. Notably, this is the first reported case of insulin autoimmune syndrome in Iran.
A 47-year-old Iranian female with no prior history of diabetes but a 10-year history of vitiligo experienced recurrent hypoglycemia symptoms as evidenced by neuroglycopenia and neurological symptoms. Biochemical evaluation revealed fasting hypoglycemia (35 mg/dL), hyperinsulinemia (> 2000 µIU/mL), elevated C-peptide (23 ng/mL), and negative sulfonylurea screening. Imaging studies excluded other causes, such as insulinoma. Considering the high level of insulin autoantibodies, the final diagnosis was insulin autoimmune syndrome. A small, frequent, low carbohydrate diet was considered as the treatment plan to relieve the hypoglycemic symptoms. The patient provided written consent authorizing the utilization of her medical data and the publication of this case study.
In patients without diabetes with autoimmune diseases, such as vitiligo, who present with hyperinsulinemic hypoglycemia accompanied by elevated C-peptide levels, insulin autoimmune syndrome can be considered as a diagnosis.
平田综合征是低血糖的一种罕见病因，其特征为胰岛素水平升高和存在胰岛素自身抗体。本病例报告旨在介绍平田综合征作为自身免疫性疾病（如白癜风）患者空腹低血糖的病因。值得注意的是，这是伊朗首例报道的胰岛素自身免疫综合征病例。
一名47岁的伊朗女性，既往无糖尿病病史，但有10年白癜风病史，出现反复低血糖症状，表现为低血糖症和神经症状。生化评估显示空腹低血糖（35mg/dL）、高胰岛素血症（>2000µIU/mL）、C肽升高（23ng/mL）以及磺脲类筛查阴性。影像学检查排除了其他病因，如胰岛素瘤。考虑到胰岛素自身抗体水平较高，最终诊断为胰岛素自身免疫综合征。采用少量、频繁、低碳水化合物饮食作为治疗方案以缓解低血糖症状。患者提供了书面同意书，授权使用其医疗数据并发表本病例研究。
对于无糖尿病的自身免疫性疾病（如白癜风）患者，若出现高胰岛素血症性低血糖且伴有C肽水平升高，可考虑诊断为胰岛素自身免疫综合征。
