Eriksson J W, Bremell T, Eliasson B, Fowelin J, Fredriksson L, Yu Z W
Department of Medicine, Umeå University Hospital, Sweden.
Diabetes Care. 1998 Aug;21(8):1217-20. doi: 10.2337/diacare.21.8.1217.
A woman born in 1949 was diagnosed in 1990 with systemic lupus erythematosus. She was treated with prednisolone, and < 1 year later she presented with marked hyperglycemia. Large doses of insulin were given four times per day. Even though the patient was thin (BMI 17.4 kg/m2), very little improvement was seen.
Serum insulin levels were high, and a euglycemic clamp investigation confirmed severe insulin resistance. The patient's serum contained insulin receptor antibodies inhibiting insulin binding, and thus the patient had a type B syndrome of insulin resistance. After diet and exercise, glycemic control stabilized and insulin treatment was withdrawn. However, in late 1993 she was in a catabolic and hyperglycemic state even though prednisolone doses were increased and azathioprin was added. In early 1994 she was treated with plasmapheresis and cyclophosphamide i.v. Subsequently, cyclosporin A was started as a maintenance therapy in addition to azathioprin. There was a rapid and sustained clinical improvement. Since late 1994 and onward, there is no sign of diabetes or glucose intolerance and there are no demonstrable insulin receptor antibodies in the patient's serum.
Severe type B insulin resistance may respond favorably to treatment with plasmapheresis and cyclophosphamide followed by cyclosporin A in combination with azathioprin.
一名1949年出生的女性于1990年被诊断为系统性红斑狼疮。她接受了泼尼松龙治疗，不到1年后出现明显的高血糖。每天注射4次大剂量胰岛素。尽管患者体型消瘦（体重指数17.4kg/m²），但血糖改善甚微。
血清胰岛素水平较高，正常血糖钳夹试验证实存在严重胰岛素抵抗。患者血清中含有抑制胰岛素结合的胰岛素受体抗体，因此该患者患有B型胰岛素抵抗综合征。经过饮食和运动干预后，血糖控制稳定，胰岛素治疗停用。然而，在1993年末，尽管增加了泼尼松龙剂量并加用了硫唑嘌呤，她仍处于分解代谢和高血糖状态。1994年初，她接受了血浆置换和静脉注射环磷酰胺治疗。随后，除硫唑嘌呤外，开始使用环孢素A作为维持治疗。临床症状迅速且持续改善。自1994年末至今，患者无糖尿病或糖耐量异常迹象，血清中也未检测到胰岛素受体抗体。
严重的B型胰岛素抵抗采用血浆置换和环磷酰胺治疗，随后联合使用环孢素A和硫唑嘌呤，可能会取得良好疗效。
