Long‐term efficacy of the sodium–glucose cotransporter 2 inhibitor, ipragliflozin, in a case of type A insulin resistance syndrome

• Main Authors: Shuichi Nagashima, Tetsuji Wakabayashi, Naoko Saito, Manabu Takahashi, Kenta Okada, Ken Ebihara, Shun Ishibashi
• Format: Article
• Language: English
• Published: Wiley 2020-09-01
• Series: Journal of Diabetes Investigation
• Subjects: Ipragliflozin; Sodium–glucose cotransporter 2 inhibitor; Type A insulin resistance syndrome
• Online Access: https://doi.org/10.1111/jdi.13241

Abstract Type A insulin resistance (IR) syndrome is a severe IR form caused by insulin receptor (INSR) gene defects. Antidiabetic drugs, including high‐dose insulin and insulin‐sensitizing agents, often fail to control associated hyperglycemia. Therapy with recombinant human insulin‐like growth factor 1 can be more effective, but it is expensive. We report a case of type A IR syndrome with an in‐frame INSR heterozygous deletion (ΔLeu999) that was treated with a combination of conventional therapy and ipragliflozin, a sodium–glucose cotransporter 2 inhibitor. Treatment reduced hemoglobin A1c levels (10.0–7.5%) and induced weight loss (54.4–52.0 kg) within 2 months, and the effects were sustained for >3 years. Sodium–glucose cotransporter 2 inhibitors might be useful to normalize blood glucose in type A IR syndrome by reducing bodyweight and ameliorating glucotoxicity.