编程死亡1（PD-1）相关的急性胰岛素依赖型糖尿病（PFD）是一种罕见的内科急性疾病，其临床特征仍不清楚。本研究旨在分析PFD患者的临床特征，以改善临床诊断和治疗。我们回顾性分析了中南大学湘雅二医院收治的10例PFD患者的临床数据，并结合相关文献中的66例患者的数据，分析和总结了他们的临床和免疫特征，并将PFD患者与不同胰岛自身抗体状态的患者进行比较。结合我们医院和文献数据，共报告76例PFD患者，年龄为（60.9±12.1）岁，男性占60.0％，身体质量指数为（22.1±5.2） kg/m2。在76例患者中，最常见的肿瘤是肺癌（43.4％）和黑色素瘤（22.4％）。在PD-1抑制剂中，最常见的药物是nivolumab（37.5％）和pembrolizumab（38.9％）。82.2％的PFD患者发生糖尿病酮症酸中毒。从PD-1相关的抑制剂治疗到高血糖的中位起病时间为95（36.0，164.5）d，发生糖尿病前的中位治疗周期为6（2.3，8.0）个周期。73例PFD患者中有26％（19/73）为阳性的胰岛自身抗体，阳性组中酮症发生的比例明显高于阴性组（100.0％ vs 75.0％， P <0.05）。在胰岛自身抗体阳性组中，PD-1抑制剂治疗后发生糖尿病的起病时间和输液次数显著低于自身抗体阴性组（28.5 d vs 120.0 d；2个周期vs 7个周期，均P <0.001）。在肿瘤免疫治疗开始后，需要警惕PFD的不良反应的发生，并且自身抗体阳性患者的PFD起病更快、更严重。检测PD-1抑制剂治疗前后的胰岛自身抗体和血糖对于早期预警和预测PFD具有重要价值。

Programmed death 1 (PD-1) associated fulminant type 1 diabetes (PFD) is a rare acute and critical in internal medicine, and its clinical characteristics are still unclear. This study aims to analyze the clinical characteristics of PFD patients to improve clinical diagnosis and treatment.We retrospectively analyzed the clinical data of 10 patients with PFD admitted to the Second Xiangya Hospital of Central South University, combined with the data of 66 patients reported in the relevant literature, analyzed and summarized their clinical and immunological characteristics, and compared the patients with PFD with different islet autoantibody status.Combined with our hospital and literature data, a total of 76 patients with PFD were reported, with the age of (60.9±12.1) years old, 60.0% male and body mass index of (22.1±5.2) kg/m2. In 76 patients, the most common tumors were lung cancer (43.4%) and melanoma (22.4%). Among PD-1 inhibitors, the most common drugs are nivolumab (37.5%) and pembrolizumab (38.9%). 82.2% of PFD patients developed diabetes ketoacidosis. The median onset time from PD-1 related inhibitor treatment to hyperglycemia was 95 (36.0, 164.5) d, and the median treatment cycle before the onset of diabetes was 6 (2.3, 8.0) cycles. 26% (19/73) of PFD patients had positive islet autoantibodies, and the proportion of ketoacidosis in the positive group was significantly higher than that in the negative group (100.0% vs 75.0%, P<0.05). The onset time and infusion times of diabetes after PD-1 inhibitor treatment in the autoantibody positive group were significantly lower than those in the autoantibody negative group (28.5 d vs 120.0 d; 2 cycles vs 7 cycles, both P<0.001).After initiation of tumor immunotherapy, it is necessary to be alert to the occurrence of adverse reactions of PFD, and the onset of PFD with islet autoantibody positive is faster and more serious than that of patients with autoantibodies negative. Detection of islet autoantibodies and blood glucose before and after treatment with PD-1 inhibitors is of great value for early warning and prediction of PFD.