Hyun Seok Choi, Byoung Ho Choi, Seok Hoo Jeong, Shung Han Choi, Dong Su Shin, Sei hyun Kim, Young Sil Eom, Sihoon Lee, Yeun Sun Kim, Ie Byung Park, Ki Young Lee
Endocrinol Metab. 2011;26(4):360-363. Published online December 1, 2011
Type B insulin resistance syndrome is rare autoimmune disease that is characterized by various abnormalities of glycemic homeostasis, from hyperglycemia caused by extreme insulin resistance to fasting hypoglycemia. It can combine with other autoimmune diseases, most commonly systemic lupus erythematosus. It usually occurs in women and accompanies acanthosis nigricans, hyperandrogenism, and, in many cases, ovary dysfunction. The diagnosis of type B insulin resistance syndrome is based largely on the presence of insulin receptor autoantibodies and hyperglycemia, or hypoglycemia and hyperinsulinemia. In some cases, patients with the type B insulin resistance have been successfully treated with immunosuppressive therapy and plasmapheresis. We experienced type B insulin resistance syndrome in a patient with chronic hepatitis B and used only plasmapheresis for treatment. The immunosuppressive therapy was omitted due to the state of activation of chronic hepatitis B. We present this case with a review of relevant literature.
BACKGROUND Insulin resistance syndrome shows extreme insulin resistance and is associated with acanthosis nigricans. We can differentiate it into type A insulin resistance that has insulin receptor defect, and type B insulin resistance due to insulin receptor autoantibody. Type B insulin resistance was firstly described by Kahn in 1976. It was often found in adult female and showed autoimmune characteristics. As clinical characteristics, there are hyperglycemia, hypoglycemia, hyperinsulinemia, extreme insulin resistance and acanthosis nigricans. METHODS: We investigated 17 insulin receptor autoantibody positive cases and 8 cases of normal control who had visited Wonju Christian Hospital from October 1994 to December 1995. Among insulin receptor autoantibody positive subject, male was 4 cases and female 13 cases. Their mean age was 42.2. We compared patients who had insulin receptor antibody positive acanthosis nigricans(IRA) with normal controls. IRA patients were 6 cases(35.3%) of nomal glucose tolerance, 11 cases(64.7%) of abnormal glucose tolerance including overt diabetes mellitus. RESULTS: The 11 cases(64.7%) among IRA patrents were obese and 13 cases(76.5%) had hyperininsulinemia. In IRA patients, mean serum insulin concentration during oral glucose tolerance test was 202.1mU/mL and it was greater than 46.3 of normal controls. Insulin sensitivity in 1.79mg/L * mM * xmU * min normal controls was higher than 0.74mg/L * mM * min of IRA patients. CONCLUSION: IRA patients showed abnormal glucose tolerance including overt diabetes millitus, severe insulin resistance, hyperinsulinemia and obesity.