Abstract：
Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) exist on a spectrum of autoimmune conditions which cause epidermal detachment and keratinocyte necrosis. Due to the rare incidence of these conditions, a dramatic heterogeneity in treatment algorithms exists. To better appreciate pharmacologic immunosuppressive therapies\' impact on survival, the authors queried a multi-institutional data network. Data for this study was extracted from TriNetX Research Network, a platform that contains ICD-9/ICD-10 coding data from a consortium of international healthcare organizations. Seventy-one institutions were queried to identify adult patients diagnosed with SJS, TEN or SJS-TEN Overlap. Cohorts were created based on the therapy received: systemic steroids (SS), diphenhydramine (DH), cyclosporine (CS), intravenous immunoglobulin (IVIG), tumor necrosis factor alpha inhibitors (TNFαi), or a combination of treatments. Cohorts were then propensity matched with patients who received supportive care. Patients who only received one of the above treatments showed no significant reduction in 90-day mortality. Patients who received CS or IVIG as part of their multitherapy showed a significantly increased risk of death when compared to supportive care (CS: RR = 1.583, 95% CI [1.119, 2.240]; IVIG: RR = 2.132, 95% CI [1.485, 3.059]). Despite their frequent utilization, this study\'s analysis suggests that none of these therapies confer significant 90-day mortality survival over supportive care alone. These results highlight the heterogeneity of therapies and emphasize the need for critical prospective appraisal of their outcomes in SJS and TEN.
摘要：
史蒂文斯-约翰逊综合征（SJS）和毒性表皮坏死松解症（TEN）存在于一系列引起表皮脱离和角质形成细胞坏死的自身免疫疾病中。由于这些情况的罕见发生，治疗算法存在巨大的异质性。为了更好地理解药物免疫抑制疗法对生存的影响，作者查询了一个多机构数据网络。本研究的数据来自TriNetX研究网络，包含来自国际医疗保健组织联盟的ICD-9/ICD-10编码数据的平台。查询了71个机构，以识别诊断为SJS的成年患者，十或SJS-TEN重叠。根据接受的治疗创建队列：全身性类固醇（SS），苯海拉明(DH),环孢菌素(CS),静脉注射免疫球蛋白（IVIG），肿瘤坏死因子α抑制剂（TNFαi），或治疗的组合。然后将队列与接受支持性护理的患者进行倾向匹配。仅接受上述治疗之一的患者90天死亡率没有显着降低。与支持治疗相比，接受CS或IVIG作为多种治疗的患者死亡风险显著增加(CS:RR=1.583,95%CI[1.119,2.240]；IVIG:RR=2.132,95%CI[1.485,3.059])。尽管他们经常使用，这项研究的分析提示,这些治疗方法均不比单独的支持治疗具有显著的90天死亡率生存率.这些结果突出了治疗的异质性，并强调了对SJS和TEN结果进行关键前瞻性评估的必要性。
