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

BACKGROUND: The 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. OBJECTIVES: To systematically assess the effectiveness of several therapies for SJS/TEN through the SCORTEN score. METHODS: Databases 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. RESULTS: Of 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. LIMITATIONS: Heterogeneity and quality of the included studies. CONCLUSIONS: Some treatments for SJS/TEN show a better performance, but there is not sufficient evidence to recommend its widespread use in all patients.

Accuracy of SCORTEN to predict the prognosis of Stevens-Johnson syndrome/toxic epidermal necrolysis: a systematic review and meta-analysis.

BACKGROUND: The SCORTEN score is a specific predictor of the probability of death for patients diagnosed with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN). OBJECTIVES: To evaluate the overall accuracy of SCORTEN and the influence of several moderators such as age, sex, geographical region and age of the study. METHODS: A systematic search was performed on MEDLINE, The Cochrane Library, EMBASE, SCOPUS and Web of Knowledge, with no restriction on language (last update 5 February 2019 for all databases). Original studies on the use of SCORTEN were eligible. The standardized mortality ratio (SMR), defined as the quotient between the number of deaths observed and the number expected following SCORTEN, was taken as the measurement of analysis. RESULTS: Sixty-four papers were part of the main meta-analysis carried out in the study. A pooled log(SMR) of -0.0889 (95% CI: -0.2023 to 0.0245) was obtained, suggesting a reasonable behaviour of SCORTEN as a predictor of mortality. The possible influence of several factors in the accuracy of SCORTEN was studied by means of meta-regression models. Multivariate meta-regression allowed finding that the mean age of the patients and the ending year of the study are the only factors that significantly influence SCORTEN predictions. The mean age of the group of patients was associated with a significant increase in the observed/expected ratio, whereas a progressive reduction in the observed/expected ratio can be appreciated over the years. Finally, an underestimation of mortality was found for SCORTEN values of 3 or less and the opposite for those above 3 (SCORTEN range: 0-7). CONCLUSIONS: The rarity of the disease and the heterogeneity of the studies included are major limitations. Despite the overall remarkable accuracy displayed by SCORTEN, the influence of several factors, as comorbidities (e.g. renal impairment), involved body surface area and patient's age, seem of enough relevance to consider a redefinition of the scale.