Ovarian stimulation toward oocyte cryopreservation for fertility preservation in a patient with Hirata syndrome: a clinical challenge in assisted reproduction.

backgroundInsulin autoimmune syndrome (IAS), or Hirata disease, is a rare autoimmune disorder characterized by the presence of autoantibodies targeting insulin, leading to episodes of postprandial hypoglycemia. First identified in Japan, the condition was historically seen primarily in the Asian population, but with global recognition and improved diagnostic tools, its prevalence has expanded. While IAS is often self-limiting and resolves with dietary modifications and discontinuation of triggering medications, its management in the context of assisted reproductive technology (ART) remains understudied. CASE PRESENTATION: This case report discusses a 28-year-old female diagnosed with IAS who underwent oocyte cryopreservation following a fertility assessment revealing low serum AMH levels. Despite a history of severe hypoglycemia, which was managed with rituximab and resolved within a month, and the presence of elevated insulin and insulin autoantibodies, she successfully completed ovarian stimulation without experiencing hypoglycemic episodes. DISCUSSION: Close monitoring of glucose levels and insulin autoantibody concentrations was essential for successful oocyte retrieval. This case underscores the importance of careful monitoring and individualized care for patients with IAS undergoing ART as autoimmune flare-ups and hypoglycemia can still occur even when the disease is in remission.

conclusionA multidisciplinary approach involving reproductive endocrinologists and fertility specialists is critical for safe management of such patients.