[Type B insulin resistance syndrome].

Area of Science:

• Endocrinology
• Immunology
• Metabolic Disorders

Background:

• Type B insulin resistance syndrome (TBIRS) is an extremely rare autoimmune condition.
• It is characterized by autoantibodies against the insulin receptor, leading to severe, treatment-resistant hyperglycemia and high mortality.
• Patients often present with significant insulin resistance shortly after the onset of autoimmune diabetes.

Purpose of the Study:

• To describe a case of TBIRS presenting in a patient with autoimmune type 1 diabetes.
• To evaluate the efficacy of glucocorticoid therapy in managing this rare condition.
• To document the patient's response to various immunosuppressive and B-cell depleting therapies.

Main Methods:

• Clinical case presentation and management.
• Biochemical monitoring of P-glucose and insulin requirements.
• Detection of insulin receptor antibodies via immunoprecipitation and binding assays.
• Treatment with glucocorticoids, plasmapheresis, rituximab, mycophenolic acid, and bortezomib.

Main Results:

• The patient experienced a >20-fold increase in insulin requirements and difficulty controlling P-glucose levels.
• Initiation of glucocorticoids led to an 80-90% reduction in insulin needs and stabilization of P-glucose to 7-8 mmol/L.
• Relapse occurred after initial remission, with transient benefits from plasmapheresis and mixed results from other immunomodulatory agents.

Conclusions:

• Glucocorticoids can be highly effective in managing Type B insulin resistance syndrome by overcoming insulin receptor blockade.
• TBIRS requires aggressive and often multi-agent immunosuppressive therapy, with variable patient responses.
• Long-term management remains challenging, potentially influenced by compliance and infection risk.