Matsushiro Mari, Haraguchi Takuya, Yamazaki Yuji, Hamamoto Yoshiyuki, Seino Yutaka
Center for Diabetes, Endocrinology and Metabolism, Kansai Electric Power Hospital, Osaka 553-0003, Japan.
Yutaka Seino Distinguished Center for Diabetes Research, Kansai Electric Power, Medical Research Institute, Kobe 650-0047, Japan.
JCEM Case Rep. 2025 Mar 20;3(4):luaf048. doi: 10.1210/jcemcr/luaf048. eCollection 2025 Apr.
IgG4-related diseases are fibroinflammatory disorders affecting multiple organs, with autoimmune pancreatitis (AIP) being a common manifestation. Steroid therapy is effective in inducing remission but has complex effects on glucose metabolism. Diabetes occurs in 40% to 80% of AIP patients, and steroids can worsen glucose tolerance, although some studies suggest they may improve pancreatic endocrine function. An 81-year-old man with elevated IgG4 levels and imaging findings consistent with AIP initially declined treatment. His condition worsened, leading to poor glycemic management and referral to our hospital. Imaging confirmed AIP and tests showed impaired insulin and glucagon secretion. He was diagnosed with pancreatic diabetes secondary to IgG4-related AIP. Initially, intensive insulin therapy was administered, but within 3 months, both insulin and glucagon secretion declined significantly in the arginine-stimulation test, necessitating steroid therapy with prednisolone (35 mg/day) for the AIP. The high dose of steroid treatment enhanced both insulin and glucagon secretion capacities but gradually declined with dose tapering. On the other hand, although steroid therapy poses a temporary risk of hyperglycemia, it likely prevented further deterioration of pancreatic endocrine function.
IgG4相关性疾病是一种影响多个器官的纤维炎性疾病，自身免疫性胰腺炎（AIP）是其常见表现。类固醇疗法可有效诱导缓解，但对糖代谢有复杂影响。40%至80%的AIP患者会发生糖尿病，类固醇会使糖耐量恶化，尽管一些研究表明它们可能改善胰腺内分泌功能。一名81岁男性，IgG4水平升高且影像学检查结果符合AIP，最初拒绝治疗。其病情恶化，导致血糖管理不佳，随后转诊至我院。影像学检查确诊为AIP，检测显示胰岛素和胰高血糖素分泌受损。他被诊断为继发于IgG4相关性AIP的胰腺性糖尿病。最初给予强化胰岛素治疗，但在3个月内，精氨酸刺激试验中胰岛素和胰高血糖素分泌均显著下降，因此需要使用泼尼松龙（35毫克/天）进行AIP的类固醇治疗。高剂量类固醇治疗增强了胰岛素和胰高血糖素的分泌能力，但随着剂量逐渐减少而逐渐下降。另一方面，尽管类固醇疗法会带来短暂的高血糖风险，但它可能防止了胰腺内分泌功能的进一步恶化。
