Matsuda Takaaki, Osaki Yoshinori, Soma Nao, Azuma Takayo, Ezura Aya, Mitani Yuta, Kikuchi Yuichi, Matsumoto Nako, Murayama Yuki, Sugano Yoko, Iwasaki Hitoshi, Mathis Bryan J, Sekiya Motohiro, Shimano Hitoshi
Department of Endocrinology and Metabolism, University of Tsukuba Hospital, Ibaraki, Japan.
Department of Endocrinology and Metabolism, Institute of Medicine, University of Tsukuba, Ibaraki, Japan.
Clin Pediatr Endocrinol. 2025 Jul;34(3):193-199. doi: 10.1297/cpe.2024-0083. Epub 2025 May 30.
Latent autoimmune diabetes in the young (LADY), also known as slowly progressive insulin-dependent diabetes mellitus (SPIDDM), is a slowly progressive form of type 1 diabetes (T1D) characterized by positive islet-related autoantibodies and, typically, an initial type 2 diabetes (T2D) phenotype. Although approximately 10% of children with T2D have positive islet-related autoantibodies, reports on the clinical course of patients with LADY are limited. We present the case of a 17-yr-old female initially diagnosed with T2D based on a body mass index (BMI) of 27 kg/m, obesity, and preserved endogenous insulin secretion. Notably, the glutamic acid decarboxylase antibody (GADA) test results fluctuated between weakly positive and negative. She developed diabetic ketosis 9 mo later, with a weak GADA titer and a high zinc transporter-8 antibody (Zn-T8A) titer, confirming autoimmune β-cell destruction consistent with T1D. Subsequent human leukocyte antigen (HLA) testing revealed the presence of the DRB115:02-DQB106:01 haplotype, which is considered protective against T1D. This case report details the clinical course of LADY, emphasizes close follow-up and re-evaluation of multiple islet-related autoantibodies in patients to distinguish LADY from T2D, and suggests that a protective HLA haplotype may have contributed to the slow onset despite high-titer Zn-T8A.
青年隐匿性自身免疫性糖尿病（LADY），也称为缓慢进展性胰岛素依赖型糖尿病（SPIDDM），是1型糖尿病（T1D）的一种缓慢进展形式，其特征是胰岛相关自身抗体呈阳性，通常最初表现为2型糖尿病（T2D）的表型。尽管约10%的T2D儿童胰岛相关自身抗体呈阳性，但关于LADY患者临床病程的报道有限。我们报告了一例17岁女性患者，最初因体重指数（BMI）为27kg/m²、肥胖且内源性胰岛素分泌保留而被诊断为T2D。值得注意的是，谷氨酸脱羧酶抗体（GADA）检测结果在弱阳性和阴性之间波动。9个月后，她出现糖尿病酮症，GADA滴度低而锌转运体8抗体（Zn-T8A）滴度高，证实存在与T1D一致的自身免疫性β细胞破坏。随后进行的人类白细胞抗原（HLA）检测显示存在DRB115:02-DQB106:01单倍型，该单倍型被认为对T1D有保护作用。本病例报告详细描述了LADY的临床病程，强调对患者进行密切随访并重新评估多种胰岛相关自身抗体以区分LADY与T2D，并表明尽管Zn-T8A滴度高，但一种保护性HLA单倍型可能导致了起病缓慢。
