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大剂量静脉注射免疫球蛋白治疗史蒂文斯-约翰逊综合征的疗效:一项回顾性多中心研究。

Effect of high-dose intravenous immunoglobulin therapy in Stevens-Johnson syndrome: a retrospective, multicenter study.

作者信息

Prins Christa, Vittorio Carmela, Padilla R Steven, Hunziker Thomas, Itin Peter, Förster John, Bröcker Eva-B, Saurat Jean-Hilaire, French Lars E

机构信息

Department of Dermatology, University School of Medicine, Geneva University Hospital, Geneva, Switzerland.

出版信息

Dermatology. 2003;207(1):96-9. doi: 10.1159/000070957.

Abstract

BACKGROUND

Stevens-Johnson syndrome (SJS) is a severe cutaneous drug reaction associated with considerable morbidity, possible transition to toxic epidermal necrolysis (TEN) and death in certain cases.

OBJECTIVE

To determine whether treatment with high-dose IVIG in SJS patients may improve outcome.

METHODS

Data from 12 patients (collected between January 1997 and November 2000 from 7 university dermatology centers in Europe and North America) diagnosed with SJS according to a recent consensus definition was analyzed retrospectively. All patients had progressive ongoing epidermal detachment at the time of treatment initiation. Patients with overlap syndromes and TEN were excluded. Tolerance, survival at 45 days after onset and total healing time were assessed.

RESULTS

Twelve SJS patients (mean age 44 years) were treated with IVIG at a mean dose of 0.6g/kg/day for an average of 4 days. An objective response to IVIG infusion was observed in all patients within a mean of 2 days, and the overall survival rate was 100%. Total skin healing occurred, on average, within 8.3 days. Time to total healing was shorter in a group of patients with fewer severe underlying diseases who had received IVIG infusion rapidly after the onset of skin lesions.

CONCLUSION

High-dose IVIG may be effective in blocking the progression of SJS and reducing the time to complete skin healing.

摘要

背景

史蒂文斯 - 约翰逊综合征(SJS)是一种严重的皮肤药物反应,伴有相当高的发病率,在某些情况下可能转变为中毒性表皮坏死松解症(TEN)并导致死亡。

目的

确定在SJS患者中使用大剂量静脉注射免疫球蛋白(IVIG)治疗是否可改善预后。

方法

回顾性分析了12例患者的数据(1997年1月至2000年11月期间从欧洲和北美的7个大学皮肤科中心收集),这些患者根据最近的共识定义被诊断为SJS。所有患者在开始治疗时均有进行性的表皮剥脱。重叠综合征和TEN患者被排除在外。评估耐受性、发病后45天的生存率和完全愈合时间。

结果

12例SJS患者(平均年龄44岁)接受IVIG治疗,平均剂量为0.6g/kg/天,平均治疗4天。所有患者在平均2天内对IVIG输注有客观反应,总生存率为100%。皮肤完全愈合平均在8.3天内发生。在一组潜在严重疾病较少且在皮肤病变发作后迅速接受IVIG输注的患者中,完全愈合时间较短。

结论

大剂量IVIG可能有效阻断SJS的进展并缩短皮肤完全愈合时间。

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