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。