症例は36歳女性。X年9月30日より37度台の発熱, 心窩部痛を自覚し10月3日より高血糖に伴う症状が出現した。10月5日未明にＡ病院に救急搬送され随時血糖1,515mg/dL, 尿ケトン体強陽性, 動脈血液分析でpH 7.120であり糖尿病性ケトアシドーシスと診断され当院に転院搬送された。入院時のHbA1cは6.3％であり, 尿中CPR 3.3μg/day, グルカゴン負荷試験において負荷前, 負荷6分後の血清CPRは0.1ng/mL以下であり, 劇症1型糖尿病と診断した。Human leukocyte antigen（HLA）は劇症1型糖尿病に疾患抵抗性のHLAであるDRB1*15:02-DQB1*06:01を保有し, 抗GAD抗体価は5.5U/mLと陽性であった。疾患抵抗性HLAを保有し抗GAD抗体陽性を示した劇症1型糖尿病の症例はわれわれの調べた範囲では既報になく貴重な症例と考えられたので報告する。

   The patient was a 36-year-old female. On September 30, X, fever (37℃ or higher) and epigastric pain were noted. On October 3, hyperglycemia-related symptoms appeared. On October 5, she was taken to Hospital A by ambulance early in the morning. The casual blood glucose level was 1,515 mg/dL, and she was markedly positive for urinary ketone bodies. Arterial blood analysis showed a pH of 7.120, leading to a diagnosis of diabetic ketoacidosis. She was referred to our hospital. On admission, the HbA1c and urinary CPR values were 6.3% and 3.3 g/day, respectively. A glucagon tolerance test showed that the serum CPR levels before loading and 6 minutes after loading were ≤0.1 ng/mL, suggesting fulminant type 1 diabetes mellitus. Concerning human leukocyte antigen (HLA), the patient had fulminant type 1 diabetes mellitus-resistant HLA, DRB1*15:02-DQB1*06:01. The GAD antibody titer was 5.5 U/mL. No study has reported any GAD-antibody-positive patient with fulminant type 1 diabetes mellitus showing disease-resistant HLA. We report this patient.