Willard Devina, Upadhyay Jagriti, Kim Chan, Steenkamp Devin
Section of Endocrinology, Diabetes, and Nutrition (D.W., J.U., D.S.), and Section of Rheumatology (C.K.), Boston Medical Center and Boston University School of Medicine, Boston, Massachusetts 02118.
J Clin Endocrinol Metab. 2016 Nov;101(11):3870-3873. doi: 10.1210/jc.2016-2146. Epub 2016 Sep 16.
Type B insulin resistance syndrome is a rare disease that occurs due to the development of autoantibodies to the insulin receptor and can result in either severe insulin resistance and hyperglycemia or, conversely, hypoglycemia. Diabetes mellitus is often severe, usually transient, and poorly responsive to exogenous insulin. Diabetic ketoacidosis is an unusual consequence of this most severe form of transient diabetes mellitus.
A 39-year-old Nigerian woman presented with significant weight loss, severe diabetic ketoacidosis, and severe insulin resistance requiring massive doses of exogenous insulin. She was diagnosed with systemic lupus erythematosus and type B insulin resistance syndrome. She was treated by immunomodulation with rituximab and pulse dose dexamethasone, and she entered euglycemic remission after 4 months of treatment. She remains independent of exogenous insulin 1 year later on maintenance azathioprine therapy.
We report a case of severe type B insulin resistance syndrome complicated by severe diabetic ketoacidosis soon after the initial diagnosis of diabetes, despite large doses of exogenous insulin therapy. Our patient achieved euglycemic remission after combination immunomodulation. This case illustrates the severe catabolic state that may occur with high anti-insulin receptor antibody titers and that combination therapy with rituximab and dexamethasone, followed by maintenance azathioprine therapy for 1 year, is an effective treatment approach for the management of type B insulin resistance syndrome.
B型胰岛素抵抗综合征是一种罕见疾病，因胰岛素受体自身抗体的产生而发病，可导致严重胰岛素抵抗和高血糖，或者相反，导致低血糖。糖尿病通常较为严重，多为短暂性，对外源性胰岛素反应不佳。糖尿病酮症酸中毒是这种最严重形式的短暂性糖尿病的罕见后果。
一名39岁的尼日利亚女性，出现显著体重减轻、严重糖尿病酮症酸中毒以及严重胰岛素抵抗，需要大剂量外源性胰岛素治疗。她被诊断为系统性红斑狼疮和B型胰岛素抵抗综合征。通过使用利妥昔单抗和冲击剂量地塞米松进行免疫调节治疗，治疗4个月后她进入血糖正常缓解期。1年后，在维持硫唑嘌呤治疗的情况下，她不再需要外源性胰岛素。
我们报告了一例在糖尿病初诊后不久即出现严重糖尿病酮症酸中毒并发症的严重B型胰岛素抵抗综合征病例，尽管给予了大剂量外源性胰岛素治疗。我们的患者在联合免疫调节治疗后实现了血糖正常缓解。该病例说明了高抗胰岛素受体抗体滴度可能导致的严重分解代谢状态，以及利妥昔单抗和地塞米松联合治疗，随后进行1年的硫唑嘌呤维持治疗，是治疗B型胰岛素抵抗综合征的有效方法。
