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Effects of treatments on the mortality of Stevens-Johnson syndrome and toxic epidermal necrolysis: A retrospective study on patients included in the prospective EuroSCAR Study

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Background

No treatment modality has been established as standard for patients with Stevens-Johnson syndrome and toxic epidermal necrolysis.

Objective

We sought to evaluate the effect of treatment on mortality in a large cohort of patients with Stevens-Johnson syndrome or toxic epidermal necrolysis.

Methods

Data on therapy were retrospectively collected from patients in France and Germany enrolled in EuroSCAR, a case-control study of risk factors.

Results

Neither intravenous immunoglobulins nor corticosteroids showed any significant effect on mortality in comparison with supportive care only. Compared with supportive care, odds ratios for death were 1.4 (95% confidence interval: 0.6-4.3) for intravenous immunoglobulins in France and 1.5 (0.5-4.4) in Germany, and 0.4 (0.1-1.7) for corticosteroids in France and 0.3 (0.1-1.1) in Germany.

Limitations

Such an observational study with retrospective data collection has obvious limitations, including heterogeneity between the countries, supportive care, treatment doses, and durations.

Conclusions

We found no sufficient evidence of a benefit for any specific treatment. The trend for a beneficial effect of corticosteroids deserves further exploration.

Section snippets

Design and data collection

EuroSCAR was designed as a European case-control study evaluating risk factors for SJS or TEN. It was conducted in 6 countries (Austria, France, Germany, Israel, Italy, and the Netherlands) between April 1997 and December 2001. Patients studied were those admitted to hospital with a diagnosis of SJS or TEN, who were actively detected in a network of about 1800 hospitals covering more than 100 million inhabitants. After obtaining informed consent, trained investigators interviewed patients,

Results

A total of 513 potential patients with SJS or TEN were enrolled in the EuroSCAR study. In all, 379 of them were patients with validated SJS and TEN, who developed the reaction outside the hospital and who were admitted because of SCAR symptoms. In all, 130 were from France, 174 from Germany, and 75 from other countries. Among the 304 patients from France and Germany, additional data on agents administered for treatment of the reaction were completed in 281 (92%) of the patients, 125 of 130 from

Discussion

From 281 patients with SJS or TEN, we retrospectively collected data on treatment and separated the patients into 3 treatment groups (IVIG, IVIG + corticosteroids, corticosteroids–in addition to supportive care) and one control group that only received supportive care.

This was an observational study with the obvious limitations inherent in this type of study for evaluating therapeutic effects. The EuroSCAR study was not conceptually designed to analyze the effect of treatments. Furthermore, the

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  • Cited by (0)

    The following institutions/companies funded the EuroSCAR project (unrestricted grants): ADIR and Cie, Bayer Pharma/AG/Vital, Boehringer Ingelheim, Cassenne, Ciba Geigy/Novartis, Cilag GmbH, Dr Willmar Schwabe, Goedecke Parke Davis, Glaxo Wellcome/GlaxoSmithKline, Hoechst AG/Hoechst Marion Roussel/Aventis, Hoffmann-La-Roche, IRIS Servier, Jouveinal Lab, LEO, LILLY, MSD Sharp and Dohme, Pfizer, Rhone Poulenc Rorer, Sanofi Winthrop/Sanofi Synthelabo GmbH, and Schering AG. Funding from pharmaceutical companies in France was managed through contract with Institut National de la Santé et de la Recherche Médicale, French Ministry of Health (PHRC AOM 98027); GIS-Institut des Maladies Rares.

    Conflicts of interest: None declared.

    Presented orally or in poster form at Journées Dermatologiques de Paris, France, December 2-6, 2003, and European Society for Dermatological Research, Austria, Vienna, September 8-9, 2004.

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