Sato N, Ohsawa I, Takagi M, Gohda T, Horikoshi S, Shirato I, Yamaguchi Y, Tomino Y
Division of Nephrology, Department of Internal Medicine, Juntendo University School of Medicine, Tokyo, Japan.
Clin Nephrol. 2010 Feb;73(2):157-62. doi: 10.5414/cnp73157.
Type B insulin resistance syndrome is a rare disease. Auto-antibodies to the insulin receptor frequently appear in the case of systemic lupus erythematosus (SLE). We report herein a case of a 56-year-old man who had presented discoid skin lesions since 1990. He was admitted to the hospital because of unconsciousness and severe hypoglycemia in 2006, and was diagnosed as having Type B insulin resistance syndrome with the presence of insulin receptor antibody. He had frequently repeated hypoglycemic and hyperglycemic episodes in spite of treatment with prednisolone (5 - 10 mg/day), and mild proteinuria of 1.5 g/day was observed. His laboratory findings on admission revealed pancytopenia and positive titer for antinuclear antibody (ANA). From these findings and his past history of skin lesions, we diagnosed him as SLE. We performed renal biopsy and his histological diagnosis was lupus nephritis Class 5 with the findings of podocytic shedding. Prednisolone dosage was increased from 10 to 60 mg/day. Thereafter, his glucose metabolism improved and proteinuria disappeared. The dose of prednisolone was tapered to 30 mg/day without recurrence of hypoglycemia and proteinuria. Early treatment with prednisolone might ameliorate proteinuria and insulin resistance. We experienced a rare case of Type B insulin resistance syndrome with increased activity of SLE, complicated with lupus nephritis. It appears that Type B insulin resistance syndrome should be suspected in differential diagnosis of hypoglycemia in SLE patients.
B型胰岛素抵抗综合征是一种罕见疾病。胰岛素受体自身抗体在系统性红斑狼疮（SLE）患者中经常出现。我们在此报告一例56岁男性患者，自1990年起出现盘状皮肤损害。2006年因意识不清和严重低血糖入院，经检查发现存在胰岛素受体抗体，被诊断为B型胰岛素抵抗综合征。尽管使用泼尼松龙（5 - 10毫克/天）治疗，他仍频繁出现低血糖和高血糖发作，并观察到轻度蛋白尿，每天1.5克。入院时实验室检查发现全血细胞减少，抗核抗体（ANA）滴度呈阳性。根据这些发现以及他过去的皮肤损害病史，我们诊断他为SLE。我们进行了肾活检，组织学诊断为5类狼疮性肾炎，伴有足细胞脱落的表现。泼尼松龙剂量从10毫克/天增加到60毫克/天。此后，他的糖代谢得到改善，蛋白尿消失。泼尼松龙剂量逐渐减至30毫克/天，未再出现低血糖和蛋白尿复发。早期使用泼尼松龙治疗可能改善蛋白尿和胰岛素抵抗。我们遇到了一例罕见的B型胰岛素抵抗综合征，伴有SLE活动增加，并合并狼疮性肾炎。在SLE患者低血糖的鉴别诊断中似乎应怀疑B型胰岛素抵抗综合征。
