Insulin autoimmune syndrome: from diagnosis to clinical management

Abstract

Autoimmune forms of hypoglycemia are a rare cause of low blood sugar levels among Caucasians, and often go misdiagnosed, exposing patients to lengthy series of pointless, potentially harmful and expensive tests. There are two types of autoimmune hypoglycemia. One is insulin autoimmune syndrome (IAS), which is characterized by hyperinsulinemic hypoglycemia, elevated insulin autoantibody (IAA) titers, no prior exposure to exogenous insulin, and no of pathological abnormalities of the pancreatic islets. This condition is also known as "Hirata's disease". The other is type B insulin resistance syndrome (TBIRS), a rare autoimmune disorder resulting in a broad array of abnormalities in glucose homeostasisfrom hypoglycemia to extremely insulin-resistant hyperglycemia-caused by the presence of insulin receptor autoantibodies (IRAbs). This review focuses on these two syndromes, describing their epidemiology, possible genetic background, clinical presentation, pathophysiology, diagnosis and treatment.

Clinical Features of Type B Insulin Resistance in Japanese Patients: Case Report and Survey-Based Case Series Study

“…The rate at which hypoglycemia triggered the diagnosis was high, and the rate of hypoglycemia was also high overall. It was indicated that hypoglycemia can be seen even in the previous report [1,2,19]. In the present survey, hypoglycemia was observed in 85.7% of Japanese cases and was considered a major symptom.…”

“…IGF-1. IGF-1 may be effective against the increased blood glucose caused by TBIR [2,6,12,19]. Considering that IGF-1 causes insulin-like action via the IGF-1 receptor in skeletal muscle, a reduction in blood glucose was expected in patients suffering from TBIR.…”

“…Type B insulin resistance (TBIR) is an autoimmune disease caused by the presence of immunoglobulin G (IgG) polyclonal antibodies, which competitively inhibit the binding of insulin to the insulin receptor [1,2]. This condition is extremely rare, and only 67 cases have been reviewed in the literature as of June 2014 [1].…”

Management of Insulin Autoimmune Hypoglycaemia: Single‐centre experience from Western India with systematic review of world literature

“…Insulin autoimmune hypoglycaemia (IAH) is characterized by autoantibodies against endogenous insulin without any prior exposure to exogenous insulin. Also known as Hirata disease, IAH is most commonly reported from Japan …”

“…Insulin auto‐antibodies (IAA) are associated with other autoimmune diseases, medications (sulfhydryl group containing), haematological disorders (Multiple Myeloma/Monoclonal Gammopathy of Undetermined Significance (MGUS), Non‐Hodgkin's B‐cell lymphoma) and infections (Hepatitis C virus) . As reported from Japan, about 80% of the cases of IAH go into spontaneous remission; but due to lack of awareness about the condition in areas outside Japan, the diagnosis and management remain challenging …”