Context: Hirata's disease (HD) is a rare autoimmune cause of hypoglycemia. Patients suffering from this condition have a genetic predisposition, determined by HLA DR4, with some differences in the worldwide population. In Caucasians HLA DRB1*0403 is the most frequent susceptibility background on which some drugs play as triggers.
Case description: We reported the case of a woman with several hypoglycemic episodes, characterized by high insulin and c-peptide levels. Biochemical and morphological exams excluded a neuroendocrine tumor. HD was diagnosed according to insulin autoantibodies positivity and patient's history, particularly about drugs taken. The HLA analysis revealed DRB1*0415 allele.
Conclusions: We found a potential new predisposing factor for HD, HLA DRB1*0415 allele, never described before as genetic background to insulin autoimmune syndrome in Caucasians.
Keywords: Alpha-lipoic acid; Hypoglycemia; Insulin autoantibodies; Insulin autoimmune syndrome.