Department of Dermatology, Peking Union Medical College, Beijing, 100730, China.
Eur J Dermatol. 2010 Nov-Dec;20(6):743-7. doi: 10.1684/ejd.2010.1077. Epub 2010 Oct 15.
Stevens-Johnson Syndrome (SJS) and Toxic epidermal necrolysis (TEN) are drug-induced diseases with a low incidence but high mortality. While there is no standard treatment, corticosteroids and intravenous immunoglobulin (IVIG) therapy have been widely used, with controversy. Our objective was to summarize the etiology and therapeutic regimen of SJS or TEN in 82 hospitalized patients in China. A retrospective study was performed on 82 patients who were diagnosed with SJS or TEN and hospitalized in Peking Union Medical College Hospital from July 1994 to August 2009. Of them, 24 were treated with IVIG plus corticosteroids (IVIG group) and the other 58 were treated with corticosteroids only (corticosteroids group). SCORTEN was used to evaluate the severity and prognosis of the patients. The efficacy of therapeutic modalities was assessed by the following parameters: starting and the maximum dose of corticosteroids, cumulative dose of corticosteroids before tapering, cumulative dose of IVIG, days of corticosteroid application before its tapering and the hospitalization days. The common agents triggering SJS/TEN in these patients were non-steroidal anti-inflammatory drugs (31 cases), anti-epileptics (18 cases), antibiotics (14 cases), antipodagrics (4 cases), sulfanilamides (4 cases) and others (11 cases), respectively. Carbamazepine was the most common drug, and induced 15 cases of SJS/TEN. The SCORTEN was significantly higher in the IVIG group than that in the corticosteroid group (2.0 ± 1.7 vs 0.8 ± 1.0, P = 0.001). Whereas no differences were observed between the two groups in the parameters including starting and maximum dose of corticosteroids, cumulative dose and the number of application days of corticosteroids before tapering and hospitalization days. However, in patients whose SCORTEN scores were 2, application of IVIG and corticosteroids shortened the duration of hospitalization from 26.4 ± 9.5 d to 18.1 ± 5.3 d (P < 0.05). No significant difference was observed in the incidence of complications between the two groups (54.2% vs 39.7%, P > 0.05). The actual mortalities were 12.5% in the IVIG group and 3.4% in corticosteroid group respectively, which were significantly lower than the predicted values (22.0% and 7.2%, respectively). Standardized mortality ratio (SMR) analysis showed a trend to a lower actual mortality (not significant) with corticosteroid treatment than the predicted mortality (SMR = 0.480; 95% CI: 0.075-1.923) and combination therapy had a tendency to reduce the mortality (not significant) rate of TEN (SMR = 0.569; 95% CI: 0.318-1.910). No significant difference in SMR was found between the two groups (P = 0.1474). Survival analysis showed that a favorable overall survival was associated with younger age (P = 0.0405). Our data indicated that early application of corticosteroids presented beneficial effects on SJS/TEN, and that combination therapy of corticosteroids and IVIG achieved a better therapeutic effect than the administration of corticosteroids alone. We recommend early treatment with IVIG at total doses of more than 2 g/kg in SJS/TEN patients whose SCORTEN are higher than 0.
史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）是药物引起的疾病，发病率低但死亡率高。虽然没有标准的治疗方法，但皮质类固醇和静脉注射免疫球蛋白（IVIG）治疗已被广泛应用，存在争议。我们的目的是总结中国 82 例住院患者 SJS 或 TEN 的病因和治疗方案。对 1994 年 7 月至 2009 年 8 月期间在北京协和医院住院诊断为 SJS 或 TEN 的 82 例患者进行回顾性研究。其中 24 例采用 IVIG 联合皮质类固醇（IVIG 组）治疗，另 58 例仅采用皮质类固醇治疗（皮质类固醇组）。SCORTEN 用于评估患者的严重程度和预后。通过以下参数评估治疗方式的疗效：皮质类固醇的起始和最大剂量、皮质类固醇减量前的累积剂量、IVIG 的累积剂量、开始减量前的皮质类固醇应用天数和住院天数。这些患者发生 SJS/TEN 的常见药物分别是非甾体抗炎药（31 例）、抗癫痫药（18 例）、抗生素（14 例）、抗痛风药（4 例）、磺胺类药（4 例）和其他（11 例）。卡马西平是最常见的药物，诱导了 15 例 SJS/TEN。IVIG 组的 SCORTEN 明显高于皮质类固醇组（2.0±1.7 vs. 0.8±1.0，P=0.001）。然而，两组患者在皮质类固醇的起始和最大剂量、累积剂量、减量前的应用天数和住院天数方面无差异。然而，对于 SCORTEN 评分为 2 的患者，应用 IVIG 和皮质类固醇可将住院时间从 26.4±9.5 天缩短至 18.1±5.3 天（P<0.05）。两组患者并发症发生率无差异（54.2% vs. 39.7%，P>0.05）。IVIG 组实际死亡率为 12.5%，皮质类固醇组为 3.4%，均明显低于预测值（分别为 22.0%和 7.2%）。标准化死亡率比（SMR）分析显示，皮质类固醇治疗的实际死亡率（无显著差异）有降低的趋势，低于预测死亡率（SMR=0.480；95%CI：0.075-1.923），联合治疗也有降低 TEN 死亡率（无显著差异）的趋势（SMR=0.569；95%CI：0.318-1.910）。两组间 SMR 无显著差异（P=0.1474）。生存分析表明，年轻患者的总体生存情况较好（P=0.0405）。我们的数据表明，皮质类固醇的早期应用对 SJS/TEN 有有益的作用，皮质类固醇和 IVIG 的联合治疗比单独使用皮质类固醇的治疗效果更好。我们建议在 SCORTEN 评分高于 0 的 SJS/TEN 患者中早期使用 IVIG，总剂量超过 2g/kg。
