史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死（TEN）存在于一系列自身免疫性疾病中，导致表皮脱离和角质形成细胞坏死。由于这些疾病的罕见发病率，治疗算法的差异性显著。为了更好地了解药物免疫抑制治疗对生存率的影响，作者查询了一个多机构数据网络。本研究的数据来自TriNetX Research Network，该平台包含了国际卫生保健组织的ICD-9/ICD-10编码数据。共查询了71个机构，以诊断为SJS、TEN或SJS-TEN重叠的成年患者为对象。根据所接受的治疗创建了不同的队列：全身性类固醇（SS）、苯海拉明（DH）、环孢素（CS）、静脉免疫球蛋白（IVIG）、肿瘤坏死因子α抑制剂（TNFαi）或治疗组合。然后，与接受支持性治疗的患者进行倾向匹配。只接受上述某种治疗的患者在90天内死亡率没有明显降低。与支持性治疗相比，接受多重治疗中的环孢素或静脉免疫球蛋白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]）。尽管这些治疗方法经常被使用，但本研究的分析表明，与仅接受支持性治疗相比，这些治疗方法都没有明显降低90天内死亡率的效果。这些结果突显了治疗方法的差异性，并强调了对SJS和TEN结果进行关键前瞻性评估的需求。Copyright © 2023 Elsevier Ltd and International Society of Burns Injuries. All rights reserved.

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.Copyright © 2023 Elsevier Ltd and International Society of Burns Injuries. All rights reserved.