出血性腦中風病人合併胰島素自身免疫綜合症：罕見案例報告與文獻回顧

背景：胰島素自體免疫綜合症候群（insulin autoimmune syndrome），又被稱為平田氏病（Hirata's disease），是一種罕見的高胰島素低血糖症的原因，過去1970至2009年間，全世界只有380位胰島素自體免疫綜合症候群的病患報告。這類疾病很容易被忽略和沒有考慮在鑑別診斷中。患者可有低血糖相關症狀如冒冷汗、頭暈、心悸、全身無力等臨床症狀。根據現有之文獻資料，出血性腦中風後合併胰島素自身免疫綜合症候群並不多見，本篇病例報告將描述這類患者的臨床表現及相關檢驗特色，並回顧關於胰島素自身免疫性綜合症候群之診斷、治療方式及預後的文獻資料。病例報告：個案為一位34歲男性，初次入住本科病房前半年發生右側基底核出血並進行頭顱鑽孔血腫抽吸手術及顱內壓偵測器置放手術，後續臨床狀況穩定後，因仍有左側肢體無力至本科接受住院復健，並於住院時有多次早晨冒冷汗及空腹低血糖紀錄。病患否認有第一型或第二型糖尿病病史，並無飲食控制或血糖用藥史，故安排糖化血色素檢驗，檢驗結果顯示糖化血色素為正常值。後續也安排75克口服葡萄糖耐量試驗（oral glucose tolerance test）來做進一步檢查，結果顯示呈現高胰島素低血糖症合併高C-胜肽（C-peptide）且病患無使用任何外源性胰島素，因此懷疑低血糖原因與胰島素自體免疫綜合症候群相關，故安排相關自體免疫疾病抗體，基因及胰島素自體抗體（insulin auto-antibody）檢驗，檢驗結果顯示胰島素自體抗體陽性反應，回顧病患外院及本院用藥史，發現在外院因病患血壓控制不佳且因有缺血性心臟病史，致輕微慢性心衰竭情況，所以在有需要時會使用卡普托利（captopril）幫忙控制血壓。根據相關文獻資料，可能卡普托利為疑似誘發胰島素自體免疫綜合症候群的藥物。我們根據文獻資料建議使用腎上腺皮質酮（prednisolone）藥物治療並且避免文獻上報導相關可能誘發胰島素自體免疫綜合症候群的藥物使用。症狀隨著藥物治療緩解，血糖保持平穩。個案持續接受復健及藥物治療後，追蹤後續無低血糖情況再發生。結論：胰島素自體免疫綜合症候群屬於造成低血糖症狀的罕見疾病，而低血糖情況在腦中風住院復健病患族群中並不少見，此個案經過多項檢驗檢查後，顯示可能原因為於外院使用卡普托利控制血壓誘發胰島素自體免疫綜合症候群。因此當住院復健病患於住院時頻繁出現空腹低血糖症狀時，且病患無相關糖尿病史時，需進步一排除相關疾病可能，並且將胰島素自體免疫綜合症候群的列為鑑別診斷，避免使用相關文獻報導的誘發藥物，若因合併有慢性心衰竭需使用血管張力素I型轉化酶抑制劑控制血壓減少心臟衰竭之再發生率或減少死亡率，我們建議需避免使用卡普托利（captopril）改由其他抗高血壓藥物作為控制血壓之藥物使用以待確認病因，並將檢驗結果作為後續治療計畫擬定之依據。

關鍵字

胰島素自體免疫綜合症候群（insulin autoimmune syndrome）；卡普托利（captopril）；低血糖症（hypoglycemia）；平田氏病（Hirata's disease）；自體免疫疾病（autoimmune disease）

並列摘要

Introduction: Insulin Autoimmune Syndrome (IAS), also known as Hirata's disease, is a rare cause of hyperinsulinemia hypoglycemia, with an overall incidence of one over one billion. Only three hundred and eightycases of IAS were reported world-wide during 1970 to 2009. Such disease was easily underestimated and misdiagnosed. Thus, we sincerely introduce a case newly diagnosed as IAS possibly induced by Angiotensin-Converting Enzyme inhibitor (ACEI) after cerebral vascular accident (CVA).The literature of insulin autoimmune syndrome was also reviewed. Case presentation: This 34-year-old male with past history of hypertension and hyperlipidemia hadsudden onset of mental drowsiness and weakness on left side limbs and was brought to emergent department (ED) for treatment on Oct 8, 2013. As Brain computed tomography (CT) showed right posterior basal ganglion hemorrhage, burrhole hematoma aspiration and intracranial pressure (ICP) monitor were performed. The postoperative course was smooth, and rehabilitation program was arranged afterward due to persistent left limbs weakness and poor walking balance. However, during admission for rehabilitation, multiple episodes of cold sweating and hypoglycemia were recorded. Even though the patient denied a history of type 1 ortype 2 diabetes mellitus and his HbA1c was normal, insulin autoimmune syndrome was suspected. The profile of autoimmune including C3, C4, rheumatic profile, dsDNA, ssDNA, C-peptide, thyroid profile (TSH, T3, free T4), insulin antibody, anti-B2 glycoprotein, anti-cardiolipin, IgG, IgG4, anti-SSa/SSb, ESR, and saliva production tests were checked. Additionally, evidence of other autoimmune diseases such as systemic lupus erythematosus, and hematological disease such as multiple myeloma, were not observed. Furthermore, the data of immune antibody showed elevated insulin antibody (82.1%). Thus, insulin autoimmune syndrome was diagnosed. The treatment of prednisolone 30mg/day was prescribed and gradually titrated to 45mg/day. As the trend of blood sugar became much more smoothly, and the patient was discharged afterward. Conclusions: Insulin autoimmune syndrome is a rare disease that causes hypoglycemia. The pathophysiology of IAS may be due to the high binding capacity and low affinity of insulin antibody(IAA), IAA can binding insulin and spontaneously dissociatelater, which inappropriately increases unbound insulin, resulting in hypoglycemic episodes. In this case, the possible cause of insulin autoimmune syndrome was induced by using captopril in previous medical treatment. Therefore, we suggest that the patient with IAS should avoid using captopril as anti-hypertensive drugs and taking medications may developing IAS which have been reported in the current literatures.

並列關鍵字

insulin autoimmune syndrome ； captopril ； hypoglycemia ； Hirata's disease ； autoimmune disease

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出血性腦中風病人合併胰島素自身免疫綜合症：罕見案例報告與文獻回顧

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