Systemic therapies for Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: a SCORTEN‐based systematic review and meta‐analysis

Abstract

BackgroundThe SCORTEN score is a specific predictor of mortality for patients with Stevens–Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN). There is little evidence in support of the common immunomodulating therapies for SJS/TEN.ObjectivesTo systematically assess the effectiveness of several therapies for SJS/TEN through the SCORTEN score.MethodsDatabases were searched for original studies on the use of SCORTEN. Six meta‐analyses were carried out on patients with SJS/TEN who received supportive care only or in combination with immunomodulating drugs: corticosteroids, cyclosporine, etanercept, immunoglobulins or a combination of corticosteroids with immunoglobulins. A multivariate meta‐regression and a network meta‐analysis were also performed.ResultsOf 3893 studies identified, fifty‐two involving 2466 patients with SJS/TEN were preselected. Data from thirty‐eight of these studies (1827 patients) were finally pooled, and results [log(SMR)] from meta‐analyses were as follows: −0.13 (95% CI, −0.42,0.16) for corticosteroids, −0.39 (95% CI, −0.87,0.09) for immunoglobulins, 0.13 (95% CI, −0.15,0.40) for supportive treatment, −0.88 (95% CI, −1.47, −0.29) for cyclosporine, −0.95 (95% CI, −1.82, −0.07) for etanercept and − 0.56 (95% CI, −0.94, −0.19) for immunoglobulins plus corticosteroids. The meta‐regression analysis confirmed that cyclosporine and immunoglobulins plus corticosteroids were associated with less deaths than predicted by SCORTEN. In the network meta‐analysis, no treatment achieved a significant reduction in the SMR.LimitationsHeterogeneity and quality of the included studies.ConclusionsSome treatments for SJS/TEN show a better performance, but there is not sufficient evidence to recommend its widespread use in all patients.

Sutureless Amniotic Membrane (ProKera®) and Intravenous Immunoglobulin in the Management of Ocular Complications of Stevens-Johnson Syndrome-Toxic Epidermal Necrolysis Overlap

“…The most commonly used systemic immunomodulatory agents in the treatment of SJS and TEN are corticosteroids, immunoglobulin, and cyclosporine. While there is strong evidence suggesting that the use of such agents is beneficial in reducing both mortality and recovery time in patients with SJS and TEN, their role in preventing ocular complications or improving visual outcomes remains questionable at best [5,21,22].…”

“…Current evidence suggests that systemic cyclosporine or the combination of corticosteroids and intravenous immunoglobulin (IVIG) in the acute phase could reduce mortality in selected patients [5]. However, until now, no evidence exists on the therapeutic benefits of systemic immunomodulatory therapy on the prevention of chronic ocular complications of SJS/TEN or the final visual outcome in such patients [4,5].…”

Intravenous Immunoglobulin Combined With Corticosteroids for the Treatment of Stevens–Johnson Syndrome/Toxic Epidermal Necrolysis: A Propensity-Matched Retrospective Study in China

“…While some retrospective studies have concluded that IVIg treatment is effective in SJS/TEN ( Metry et al, 2003 ; Morici et al, 2000 ; Prins et al, 2003a ; Prins et al, 2003b ; Trent et al, 2003 ), and recent meta-analyses have shown that high-dose IVIg (≥2 g/kg) has a beneficial effect in decreasing the mortality of SJS/TEN ( Barron et al, 2015 ), a retrospective study of IVIg treatment suggested that IVIg did not significantly improve survival both at high (≥3 g/kg) and low (<3 g/kg) dosages ( Lee et al, 2013 ). Furthermore, the latest meta-analyses and systematic reviews have demonstrated that IVIg was not associated with a promising survival benefit ( Torres-Navarro et al, 2020b ; Zimmermann et al, 2017 ). However, plenty of studies indicated that IVIg combined with corticosteroids provides a survival benefit.…”

Recent Dermatological Treatments for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Japan

“…Recently, a significantly lower mortality rate in the steroid treatment group than the mortality rate predicted by the SCORTEN has been reported ( 14 ). Furthermore, there are multiple previous studies that showed the possible usefulness of combination therapy with corticosteroids and IVIG as mentioned above ( 15 , 26 – 28 , 33 , 38 ), even though steroid use alone did not show sufficient effects in those studies. Therefore, the therapeutic effect of steroids remains controversial.…”