Insulin autoimmune syndrome

Insulin Autoimmune Syndrome

Insulin Autoimmune Syndrome (IAS), also known as Hirata's disease, is a rare autoimmune disease characterized by the presence of autoantibodies against endogenous insulin. This condition can lead to episodes of spontaneous hypoglycemia due to the binding of insulin by these autoantibodies, which interferes with normal insulin regulation.

History

IAS was first described by Dr. Yoshio Hirata in Japan in 1970. Since then, it has been reported primarily in East Asia, although cases have been identified worldwide.

Pathophysiology

The pathophysiology of IAS involves the production of autoantibodies against insulin. These autoantibodies can bind to insulin, forming immune complexes that delay the clearance of insulin from the bloodstream. This can result in prolonged periods of hyperinsulinemia followed by episodes of hypoglycemia when the insulin is suddenly released from the immune complexes.

Clinical Presentation

Patients with IAS typically present with recurrent episodes of hypoglycemia, which can occur spontaneously or after meals. Symptoms of hypoglycemia may include sweating, palpitations, tremors, confusion, and in severe cases, seizures or loss of consciousness.

Diagnosis

The diagnosis of IAS is based on the clinical presentation and the detection of insulin autoantibodies in the blood. Other causes of hypoglycemia, such as insulinoma or exogenous insulin administration, must be ruled out. Laboratory tests may show elevated levels of insulin and C-peptide during hypoglycemic episodes.

Treatment

Management of IAS involves addressing the hypoglycemia and reducing the production of insulin autoantibodies. Initial treatment may include dietary modifications to prevent hypoglycemia. In some cases, medications such as glucocorticoids or immunosuppressive drugs may be used to reduce autoantibody production. Plasmapheresis has also been used in severe cases to remove autoantibodies from the blood.

Prognosis

The prognosis for patients with IAS is generally good, especially with appropriate management. Many patients experience a reduction in symptoms over time, and some may achieve complete remission.

Related Pages

• Autoimmune disease
• Hypoglycemia
• Insulin
• Insulinoma
• Glucocorticoid
• Immunosuppressive drug
• Plasmapheresis