A case report of type B insulin resistance syndrome with multisystem immune impairment

This report details the diagnosis and management of a patient with type B insulin resistance syndrome(B-IRS) complicated by multisystem immune impairment. B-IRS is a rare autoimmune disorder often presenting with abnormal blood glucose fluctuations and may coexist with other autoimmune conditions. This 40-year-old male patient initially presented with hyperglycaemia, followed by alternating hyper- and hypoglycaemia. Hypoglycaemia persisted after discontinuing hypoglycaemic agents. Subsequent follow-up revealed three additional autoimmune diseases: Sjögren′s syndrome, Hashimoto′s thyroiditis, and PLA2R-negative membranous nephropathy. Diagnosis was confirmed via oral glucose tolerance test(OGTT) with concurrent insulin/C-peptide release testing and polyethylene glycol(PEG) precipitation analysis. Combination therapy with corticosteroids and immunomodulators led to clinical improvement. This article suggests that when a patient presents with severe insulin resistance, symptomatic hyperglycemia, life-threatening hypoglycemia, and is accompanied by multiple autoimmune diseases, a high index of suspicion for type B insulin resistance syndrome should be maintained.