Toxic epidermal necrolysis: Does immunoglobulin make a difference?
Toxic epidermal necrolysis: Does immunoglobulin make a difference?
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DOI：
10.1097/01.bcr.0000105096.93526.27
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发表时间：
2004-01-01
期刊：
影响因子：
--
通讯作者：
Gamelli, RL
中科院分区：
文献类型：
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作者：
Brown, KM;Silver, GM;Gamelli, RL
实验证据表明Fas配体介导的角质形成细胞凋亡是中毒性表皮坏死松解综合征（TEN）的潜在机制。体外研究表明，免疫球蛋白（IG）治疗在阻断Fas配体信号传导中具有潜在作用，从而降低TEN的严重程度。轶事报道描述了使用IG成功治疗TEN患者;然而，迄今为止，没有研究使用机构对照分析了使用IG治疗的大量患者的结局数据。SCORTEN疾病严重程度评分使用年龄、心率、TBSA斯劳、恶性肿瘤史和入院血尿素氮、血清碳酸氢盐和葡萄糖水平对TEN患者的严重程度进行分级并预测预后。进行了一项回顾性病历审查，包括自1997年以来在我们烧伤中心接受TEN治疗的所有患者。从2000年1月开始，对所有经活检证实的TEN患者进行IG治疗。21名十岁患者在接受IG治疗之前接受了治疗（无IG组），24名患者已接受了IG治疗。收集SCORTEN数据，以及住院时间（LOS）和出院时的状态。每例患者的SCORTEN评分为0 - 6分，年龄大于40岁、TBSA斯劳大于10%、恶性肿瘤史、入院时BUN大于28 mg/dl、HCO 3小于20 mg/dl和葡萄糖大于252 mg/dl各得1分。比较了接受IG和未接受IG治疗的患者的结局。IG治疗前患者的总死亡率为28.6%（6/21），IG治疗后患者的总死亡率为41.7%（10/24）。年龄和TBSA斯劳无显著差异。两组之间的平均SCORTEN相当（无IG组为2.2，IG组为2.7，P = 0.3），没有任何SCORTEN评分的患者组显示出从IG治疗中显著获益。IG组的总体LOS以及存活者的LOS更长。该系列代表了IG治疗后TEN患者结局的最大单机构分析。我们的数据没有显示任何严重程度的IG治疗的TEN患者的死亡率有显著改善，可能表明使用IG的潜在危害。在临床试验之外，不应给予TEN患者IG。一项多中心、前瞻性、双盲随机试验是必要的，迫切需要确定IG治疗在TEN患者的护理中是有益还是有害。
Experimental evidence implicates Fas ligand-mediated keratinocyte apoptosis as an underlying mechanism of toxic epidermal necrolysis syndrome (TEN). In vitro studies indicate a potential role for immunoglobulin (Ig) therapy in blocking Fas ligand signaling, thus reducing the severity of TEN. Anecdotal reports have described successful treatment of TEN patients with Ig; however, no study to date has analyzed outcome data in a large series of patients treated with Ig using institutional controls. The SCORTEN severity-of-illness score ranks severity and predicts prognosis in TEN patients using age, heart rate, TBSA slough, history of malignancy, and admission blood urea nitrogen, serum bicarbonate, and glucose levels. A retrospective chart review was performed that included all patients treated for TEN at our burn center since 1997. Ig therapy was instituted for all patients with biopsy-proven TEN beginning in January 2000. Twenty-one TEN patients were treated before Ig (no-Ig group), and 24 patients have been treated with Ig. SCORTEN data were collected, as well as length of stay (LOS) and status upon discharge. Each patient was given a SCORTEN of 0 to 6, with 1 point each for age greater than 40, TBSA slough greater than 10%, history of malignancy, admission BUN greater than 28 mg/dl, HCO3 less than 20 mg/dl, and glucose greater then 252 mg/dl. Outcome was compared between patients treated with Ig and without Ig. Overall mortality for patients treated before Ig was 28.6% (6/21), and with Ig, mortality was 41.7%% (10/24). There was no significant difference in age or TBSA slough. The average SCORTEN between the groups was equivalent (2.2 in no-Ig group vs 2.7 in Ig group, P = 0.3), and no group of patients with any SCORTEN score showed a significant benefit from Ig therapy. Overall LOS as well as LOS for survivors was longer in the Ig group. This series represents the largest single-institution analysis of TEN patient outcome after institution of Ig therapy. Our data do not show a significant improvement in mortality for TEN patients treated with Ig at any level of severity and may indicate a potential detriment in using Ig. Ig should not be given to TEN patients outside of a clinical trial. A multicenter, prospective, double-blinded randomized trial is necessary and urgently indicated to determine whether Ig therapy is beneficial or harmful in the care of TEN patients.