Type B Insulin-resistance syndrome: a cause of reversible autoimmune hypoglycaemia

Rituximab for the treatment of type B insulin resistance syndrome: a case report and review of the literature

“…Previous articles have reported that type B insulin resistance syndrome is frequently associated with autoimmune diseases, and that systemic lupus erythematosus was the most common underlying disease (62.69%) [4]. Table 1 summarizes the case reports, which include 11 people (four men and seven women) with type B insulin resistance syndrome who were treated with rituximab [3][4][5][6][7]. Corticosteroid treatment is standard for type B insulin resistance syndrome, and cyclophosphamide, plasmapheresis and intravenous immunoglobulin infusion are other options to reduce antiinsulin receptor antibodies.…”

“…People with type B insulin resistance syndrome have been treated with immunosuppressive therapy, such as corticosteroids, cyclophosphamide and cyclosporine; however, a standardized treatment regimen for type B insulin resistance syndrome has yet to be established. Since that time, several cases of type B insulin resistance syndrome effectively treated with rituximab have been reported [3][4][5][6]. Since that time, several cases of type B insulin resistance syndrome effectively treated with rituximab have been reported [3][4][5][6].…”

“…Coll et al [3] first reported that rituximab, an anti-CD20 monoclonal antibody, had a striking effect on high insulin resistance in a person with type B insulin resistance syndrome by reducing anti-insulin receptor antibodies. Since that time, several cases of type B insulin resistance syndrome effectively treated with rituximab have been reported [3][4][5][6]. By contrast, Takei et al [7] recently reported a rituximab-resistant case of type B insulin resistance syndrome.…”

The insulin autoimmune syndrome (IAS) as a cause of hypoglycaemia: an update on the pathophysiology, biochemical investigations and diagnosis

“…In conclusion, new informative cases of IAS in Caucasians continued to appear in the world literature [27,28,69,70,75,[90][91][92][93][94][95][96][97][98][99]. The very wide range of their clinical presentations and biochemical findings re-emphasised that IAS is a highly heterogeneous syndrome.…”

“…The postulate is that the binding of agonistic antibodies to receptors inhibits endogenous insulin binding, thus reducing its removal by endocytosis and subsequent intracellular metabolic degradation [67] resulting in hyperinsulinaemia and concomitant hypoglycaemia with inverse C-peptide/insulin ratio (normally C-peptide concentration in blood is ~5 times higher than insulin) [68]. The first case of insulin receptor antibodies was reported in 1976 [68] with new variants still being described [69][70][71][72]. Reported hypoglycaemic manifestations vary occurring as fasting, postprandial or spontaneous and in some can be severe and protracted.…”