JMIR Publications

ABSTRACT

Steven-Johnson Syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome are a spectrum of life-threatening conditions characterized by sloughing of the skin and epidermal necrosis. SJS/TEN generally occurs after initiation of a new medication and lead to infection, electrolyte imbalances, multi-organ failure and death. Despite the mortality risk, there is a lack of consensus in the acute management of SJS/TEN, with systemic interventions and supportive measures being the general course of treatment. This manuscript aims to summarize the key findings from the original Cochrane systematic review of interventions for treatment of SJS, TEN and SJS/TEN overlap syndrome. To evaluate the systemic therapies for SJS/TEN, a systematic review of clinical trials and prospective observational comparative studies of SJS/TEN was conducted. The primary endpoint was disease specific mortality (DSM) and adverse events (AE's) leading to discontinuation of the systemic treatment therapy. Secondary endpoints included time to complete re-epithelialization, intensive care unit length of stay, total hospital length of stay, illness sequelae, and AE's. Key comparator studies across different treatment options were also included to highlight the optimal agent for SJS/TEN treatment. Nine total studies were identified and included in the systematic review. Systemic corticosteroids had a higher risk of DSM compared to other therapeutic agents. Corticosteroids and IVIG compared to IVIG alone showed no difference in risk of DSM, time to re-epithelialization, and length of stay. The patients receiving etanercept were 49% less likely to have DSM compared to prednisolone. Serious adverse events (SAE's) such as sepsis and respiratory failure occurred with etanercept and corticosteroids, but it was unclear whether these AE’s led to treatment discontinuation. In the cyclosporine versus IVIG comparator group, it was uncertain if there was a difference in mortality. Future studies including larger clinical trials are needed to evaluate direct comparisons between different treatment agents to optimize agent selection for SJS/TEN management.