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静脉注射免疫球蛋白在中毒性表皮坏死松解症中的作用:专门中心治疗的 64 例患者的回顾性分析。

The role of intravenous immunoglobulin in toxic epidermal necrolysis: a retrospective analysis of 64 patients managed in a specialized centre.

机构信息

Department of Dermatology, Singapore General Hospital, Outram Road, Singapore, 169608.

出版信息

Br J Dermatol. 2013 Dec;169(6):1304-9. doi: 10.1111/bjd.12607.

Abstract

BACKGROUND

Toxic epidermal necrolysis (TEN) is a severe cutaneous adverse drug reaction with a mortality of 40%. Intravenous immunoglobulin (IVIg) is widely used as a specific treatment for this reaction, although evidence of its benefit is conflicting.

OBJECTIVES

We sought to evaluate whether the use of IVIg improved mortality in patients with Stevens-Johnson syndrome (SJS)/TEN overlap and TEN.

METHODS

We retrospectively analysed data for 64 patients with SJS/TEN overlap and TEN who were treated with IVIg at a single referral centre. The primary outcome analysed was in-hospital mortality. Predicted mortality was calculated based on severity-of-illness score for TEN (SCORTEN) values. Secondary analyses of survival based on IVIg dosages and prior corticosteroid exposure were also performed.

RESULTS

There were 28 cases of SJS/TEN overlap and 36 cases of TEN, with a mean SCORTEN value of 2·6. The mean dose of IVIg given was 2·4 g kg(-1) and the mean delay from the onset of epidermal detachment to administration of IVIg was 3·2 days. There were 20 deaths (31%) in our cohort. The standardized mortality rate was 1·10 (95% confidence interval 0·62-1·58). Subgroup analysis comparing survivors and nonsurvivors showed a higher SCORTEN in nonsurvivors (3·4 vs. 2·2). There were no differences with regard to the dosage, delay and duration of IVIg administration. When stratified according to dosage, there was no mortality difference between patients who receive high-dose (≥ 3 g kg(-1) ) vs. low-dose (< 3 g kg(-1) ) IVIg.

CONCLUSIONS

This study shows that the use of IVIg does not yield survival benefits in SJS/TEN overlap and TEN, even when corrected for IVIg dosages.

摘要

背景

中毒性表皮坏死松解症(TEN)是一种严重的药物不良反应,死亡率为 40%。静脉注射免疫球蛋白(IVIg)被广泛用作治疗这种反应的特效药,尽管其疗效存在争议。

目的

我们旨在评估 IVIg 是否能改善 Stevens-Johnson 综合征(SJS)/TEN 重叠和 TEN 患者的死亡率。

方法

我们对在一个单一转诊中心接受 IVIg 治疗的 64 例 SJS/TEN 重叠和 TEN 患者的数据进行了回顾性分析。分析的主要结果是住院死亡率。根据 TEN 严重程度评分(SCORTEN)值预测死亡率。还进行了基于 IVIg 剂量和皮质类固醇暴露史的生存的次要分析。

结果

有 28 例 SJS/TEN 重叠,36 例 TEN,平均 SCORTEN 值为 2.6。给予的 IVIg 平均剂量为 2.4 g/kg,从表皮脱落开始至给予 IVIg 的平均延迟时间为 3.2 天。我们的队列中有 20 例死亡(31%)。标准化死亡率为 1.10(95%置信区间 0.62-1.58)。比较幸存者和非幸存者的亚组分析显示,非幸存者的 SCORTEN 更高(3.4 比 2.2)。在 IVIg 剂量、延迟和持续时间方面没有差异。按剂量分层时,接受高剂量(≥3 g/kg)和低剂量(<3 g/kg)IVIg 的患者之间死亡率无差异。

结论

本研究表明,即使考虑到 IVIg 剂量,IVIg 在 SJS/TEN 重叠和 TEN 中也不能带来生存获益。

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