A clinical case of insulin autoimmune syndrome with monoclonal gammopathy of uncertain significance; complexity in management.

胰岛素自身免疫综合征的临床病例 ， 具有不确定意义的单克隆丙种球蛋白病 ； 管理的复杂性。

• 发表时间: 2024 Jun
• 来源期刊: Oxf Med Case Reports
• DOI: 10.1093/omcr/omae054
• PMID: 38860020
• 文章类型: Case Reports
• 作者列表: Clifford L,Joseph F,Joshi T

关键词: C-peptide; MGUS; anti-insulin antibodies; hypoglycaemia; insulin; insulin autoimmune syndrome

Abstract

Insulin autoimmune syndrome (IAS) is a rare cause of spontaneous hypoglycaemia. We discuss a 91-year-old Caucasian lady who presented with syncope and episodic adrenergic and neuroglycopenic symptoms. Despite significantly elevated insulin, C-peptide, and proinsulin levels with the presence of anti-insulin antibodies, a pancreatic mass was not identified. Serum immunoelectrophoresis demonstrated monoclonal gammopathy of undetermined significance (MGUS). Treatment involved high-dose steroids, diazoxide, corn starch and acarbose, however the patient passed away four months later due to worsening co-morbidities. The management of IAS in the setting of MGUS is challenging.

摘要

胰岛素自身免疫综合征（IAS）是自发性低血糖的罕见原因。我们讨论了一位91岁的白种人女士，她出现了晕厥和发作性肾上腺素能和神经糖减少症状。尽管胰岛素显著升高，C-肽，和胰岛素原水平与抗胰岛素抗体的存在，未发现胰腺肿块.血清免疫电泳显示了意义不明的单克隆丙种球蛋白病（MGUS）。治疗涉及高剂量类固醇，二氮嗪,玉米淀粉和阿卡波糖,然而，由于并发症恶化，患者在四个月后去世。MGUS设置中的IAS管理具有挑战性。