Type B insulin resistance syndrome induced by systemic lupus erythematosus and successfully treated with intravenous immunoglobulin: case report and systematic review

Abstract

Type B insulin resistance syndrome is characterized by the formation of autoantibodies against insulin receptors, which can cause severe hyperglycemia and insulin resistance. Systemic lupus erythematosus is the most common underlying diseases of the syndrome. This report details our study of a case involving a Chinese female with type B insulin resistance syndrome as well as systemic lupus erythematosus who completely recovered after undergoing immunosuppressive therapy, specifically pulse therapy utilizing intravenous immunoglobulin. We also conducted search in MEDLINE and Chinese BioMedicine database to identify relevant literatures published in the past 46 years. From our searches, six case reports in Chinese, 15 case reports, and a 28-year perspective article in English met our criteria; a total of 67 cases were included in our report. The mean age of subjects at presentation for groups A, B, and C were 42.95, 44.10, and 41.68 years, respectively, yielding no significant difference between these groups. African Americans were the most susceptible group to type B insulin resistance syndrome, followed by Asians representing 20.90 % of all cases. Comparisons between the three main racial groups surveyed indicated that the mean age of subjects at presentation were very contiguous for African Americans and Asians, and mean age of white people was remarkably higher than either of the first two groups. The syndrome appeared most common among Asian males, and white males were relatively less likely to suffer from type B insulin resistance syndrome. Hypoglycemia was most commonly observed in white people than in other racial groups. Hypoalbuminemia, elevated serum immunoglobulin G, and elevated sedimentation rates were more common in African Americans; Asian cases were more likely to show low serum C3 or C4 and nephritis. Two cases received intravenous immunoglobulin therapy, which has a remarkably rapid effect on insulin resistance.

Successful treatment of type B insulin resistance with mixed connective tissue disease by pulse glucocorticoids and cyclophosphamide

“…Treatment for the underlying autoimmune condition is critical for both primary disease and refractory hyperglycemia. The most commonly used therapeutic intervention for underlying disease includes steroids, cyclophosphamide, plasmapheresis and intravenous immunoglobulin. In 2010, a group at the National Institutes of Health (USA) published the largest case series in which a new treatment protocol with rituximab was tested in their patient population, and it was reported to be effective in one case from Europe.…”

Efficacy of oral glucocorticoid and cyclosporine in a case of rituximab‐refractory type B insulin resistance syndrome

“…Previously reported treatments for type B insulin resistance syndrome include recombinant human insulin-like growth factor-1 (IGF-1), plasmapheresis, immunoglobulin G (IgG), and various combinations of immunosuppressants2–4,6–10. Based on more than 30 years of expert experience, Malek et al 3.…”

“…of the NIH notably advocated the use of combination therapy with rituximab, pulse steroids, and immunosuppressive drugs, including cyclophosphamide, cyclosporine, and azathioprine. In addition, Zhang et al 10. also reported a case that was successfully treated with a combination of intravenous cyclophosphamide, methylprednisolone, and IgG.…”

“…Nevertheless, in the present case, rituximab therapy was ineffective. Previously reported treatments for type B insulin resistance syndrome include recombinant human insulin-like growth factor-1 (IGF-1), plasmapheresis, immunoglobulin G (IgG), and various combinations of immunosuppressants [2][3][4][6][7][8][9][10] . Based on more than 30 years of expert experience, Malek et al 3 of the NIH notably advocated the use of combination therapy with rituximab, pulse steroids, and immunosuppressive drugs, including cyclophosphamide, cyclosporine, and azathioprine.…”

“…Based on more than 30 years of expert experience, Malek et al 3 of the NIH notably advocated the use of combination therapy with rituximab, pulse steroids, and immunosuppressive drugs, including cyclophosphamide, cyclosporine, and azathioprine. In addition, Zhang et al 10 also reported a case that was successfully treated with a combination of intravenous cyclophosphamide, methylprednisolone, and IgG. Unfortunately, these reports of highly efficient protocols for treating type B insulin resistance syndrome with fewer adverse effects were not available when our treatment began in April 2010.…”