A retrospective analysis of mortality risk and immunosuppressive therapy for Stevens-Johnson Syndrome and toxic epidermal necrolysis syndrome using the TriNetX research network

• Published in: Burns 2024-02, Vol.50 (1), p.75-86
• Main Authors: Ozhathil, Deepak K., Powell, Carter M., Corley, Caroline V., Golovko, George, Song, Juquan, El Ayadi, Amina, Wolf, Steven E., Kahn, Steven A.
• Format: Article
• Language: English
• Subjects: Adult; Burns - complications; Cyclosporin; Cyclosporine - therapeutic use; Humans; Immunoglobulins, Intravenous - therapeutic use; Immunosuppression Therapy - adverse effects; IVIG; Prospective Studies; Retrospective Studies; SJS; SJS-TEN Overlap syndrome; Steroids; Stevens-Johnson Syndrome; Stevens-Johnson Syndrome - drug therapy; Stevens-Johnson Syndrome - etiology; TEN; TENS; TNF-alpha inhibitors; Toxic Epidermal Necrolysis; TriNetX
• ISSN: 0305-4179; 1879-1409; 1879-1409
• DOI: 10.1016/j.burns.2023.08.009

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. •Literature on SJS and TEN is limited to small cohort single institution trials.•Treatment options: immunosuppressive medications vs. supportive care alone.•This trial found pharmacologic therapies had worse outcomes than supportive care alone.•Cyclosporin and IVIG may increased risk of mortality.•Caution is advised with clinical application of current pharmacologic recommendations.