Cyclosporin for the treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN): a systematic review of observational studies and clinical trials focusing on single therapy, combination therapy, and comparative assessments.

Abstract

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, severe, and potentially life-threatening skin and mucous membrane disorders. They are characterized by widespread skin and mucosal detachment and necrosis, and are classified based on the percentage of total body surface area (TBSA) affected. Given the severe and often life-threatening nature of these conditions, the identification and implementation of effective treatments is crucial. Notably, cyclosporin has demonstrated efficacy in managing these challenging conditions.

Methods: A systematic search was carried out through the PubMed, Scopus, Embase, Web of Science, and Cochrane Library databases until May 2024. Additionally, a manual search was conducted through the reference lists of the included studies to minimize the risk of missing reports.

Results: Overall, 17 studies involving 4761 patients were included in our analysis. The majority of the included studies suggested favorable outcomes for the use of cyclosporin in SJS/TEN patients. The use of cyclosporin was associated with improved survival rates, early arrest of disease progression, faster re-epithelialization, reduced length of hospital stays, and lower rates of multi-organ failure. However, a few studies did not find a survival advantage with cyclosporin and even reported an increased risk of mortality, as well as an increased TBSA detachment and risk of infection.

Conclusion: Most studies indicate positive outcomes with cyclosporin treatment in SJS/TEN patients. This is likely due to cyclosporin's immunomodulatory properties, which may help attenuate the severe inflammatory response associated with these conditions.