Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
J Invest Dermatol. 2018 Nov;138(11):2315-2321. doi: 10.1016/j.jid.2018.04.027. Epub 2018 Jul 27.
Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare, severe mucocutaneous reaction with few large cohorts reported. This multicenter retrospective study included patients with SJS/TEN seen by inpatient consultative dermatologists at 18 academic medical centers in the United States. A total of 377 adult patients with SJS/TEN between January 1, 2000 and June 1, 2015 were entered, including 260 of 377 (69%) from 2010 onward. The most frequent cause of SJS/TEN was medication reaction in 338 of 377 (89.7%), most often to trimethoprim/sulfamethoxazole (89/338; 26.3%). Most patients were managed in an intensive care (100/368; 27.2%) or burn unit (151/368; 41.0%). Most received pharmacologic therapy (266/376; 70.7%) versus supportive care alone (110/376; 29.3%)-typically corticosteroids (113/266; 42.5%), intravenous immunoglobulin (94/266; 35.3%), or both therapies (54/266; 20.3%). Based on day 1 SCORTEN predicted mortality, approximately 78 in-hospital deaths were expected (77.7/368; 21%), but the observed mortality of 54 patients (54/368; 14.7%) was significantly lower (standardized mortality ratio = 0.70; 95% confidence interval = 0.58-0.79). Stratified by therapy received, the standardized mortality ratio was lowest among those receiving both steroids and intravenous immunoglobulin (standardized mortality ratio = 0.52; 95% confidence interval 0.21-0.79). This large cohort provides contemporary information regarding US patients with SJS/TEN. Mortality, although substantial, was significantly lower than predicted. Although the precise role of pharmacotherapy remains unclear, co-administration of corticosteroids and intravenous immunoglobulin, among other therapies, may warrant further study.
史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症（SJS/TEN）是一种罕见的严重黏膜皮肤反应，仅有少数大样本队列研究报道。这项多中心回顾性研究纳入了美国 18 家学术医疗中心的住院会诊皮肤科医生诊治的 SJS/TEN 成年患者。2000 年 1 月 1 日至 2015 年 6 月 1 日期间共纳入 377 例 SJS/TEN 成年患者，其中 260 例（69%）来自 2010 年以后。SJS/TEN 最常见的病因是药物反应，377 例患者中有 338 例（89.7%），最常见的药物是甲氧苄啶/磺胺甲恶唑（89/338；26.3%）。大多数患者在重症监护病房（100/368；27.2%）或烧伤病房（151/368；41.0%）接受治疗。大多数患者接受了药物治疗（266/376；70.7%）而非单纯支持治疗（110/376；29.3%）——通常为皮质类固醇（113/266；42.5%）、静脉注射免疫球蛋白（94/266；35.3%）或两种疗法（54/266；20.3%）。根据第 1 天 SCORTEN 预测死亡率，预计有 78 例院内死亡（77.7/368；21%），但实际死亡 54 例（54/368；14.7%）显著低于预期（标准化死亡率比=0.70；95%置信区间=0.58-0.79）。分层分析治疗方法，接受皮质类固醇和静脉注射免疫球蛋白联合治疗患者的标准化死亡率最低（标准化死亡率比=0.52；95%置信区间 0.21-0.79）。这项大型队列研究提供了美国 SJS/TEN 患者的当代信息。尽管死亡率相当高，但实际死亡率明显低于预测值。尽管确切的药物治疗作用仍不清楚，但皮质类固醇和静脉注射免疫球蛋白联合等疗法可能值得进一步研究。
