CHICAGO – , compared with other treatment modalities, a systematic review suggested.
Although rare in the pediatric population – with an incidence of approximately 1 case in 2 million – Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are both life-threatening mucocutaneous reactions.
Doug Brunk
Danny Mansour
Because reliable and validated data in the management of these conditions are lacking, he and his associates performed a systematic review to evaluate the effectiveness of reported treatment modalities using specific outcome measures.
“This study is unique in that it aims to not only capture more commonly employed interventions such as supportive care, surgical debridement, intravenous immunoglobulin [IVIG], and corticosteroids but also newer modalities that coincide with our improved understanding of the pathogenesis of the disease, such as the use of cyclosporine and biologics,” said Mr. Mansour, a third-year medical student at the University of Toronto.
The researchers systematically reviewed English and non-English articles using EMBASE, MEDLINE, the Cochrane Central Register of Controlled Trials, the Cochrane Database of Systematic Reviews, the Database of Abstracts of Reviews of Effects, the NHS Economic Evaluation Database, and the Health Technology Assessment Database. They excluded nonpediatric cases and those in which specific treatment modalities and outcome measures of interest were missing. In all, 302 articles were included in the study – 197 that examined mortality and 105 that examined time for arrest of progression of blistering, re-epithelialization, and length of hospital stay.
The main treatment modalities included supportive care alone, IVIG, corticosteroids, cyclosporine, surgical debridement, and biologics. The average time for arrest of progression of blistering was 8.4 days for supportive care, 4 days for IVIG, 6 days for corticosteroids, 2.5 days for cyclosporine, and 1 for infliximab. No data were available for time to arrest of progression for surgical debridement alone.
The average time for re-epithelialization was 24 days for supportive care, 18 days for surgical debridement, 8.7 days for IVIG, 12.2 days for corticosteroids, 10.6 days for cyclosporine, and 13 days for infliximab. Average length of hospital stay varied from 15.6 days for supportive care, 24.3 days for surgical debridement, 17.4 days for IVIG, 11.5 for corticosteroids, 15.5 for cyclosporine, and 26.3 for biologics.
The overall mortality was 4.6% among those who received supportive care alone, 8.3% among those who received IVIG, 4.5% among those who received corticosteroids, and 0.9% among those who received IVIG plus corticosteroids. Specifically, the rates of mortality for SJS and TEN cases treated with supportive care alone were 2% for SJS cases and 15.3% for TEN cases, respectively. The rates for other treatment modalities were 3.1% and 11% for IVIG, 1.7% and 9.3% for corticosteroids, and 0% and 2.1% for IVIG plus corticosteroids.
“While it was not surprising that mortality rates in pediatric SJS and TEN are low, the rates were slightly higher than anticipated,” Mr. Mansour said. “In addition, our data show that, although mortality is not influenced by certain therapeutic interventions in SJS patients, it may play a role in the management of the more severe disease, TEN.”
He acknowledged certain limitations of the study, including the fact that many of the data were derived from small-cohort retrospective studies and isolated case reports, giving rise to data heterogeneity and publication bias. “In addition, the mortality data we gathered may not necessarily represent patient survival, as many studies had variable follow-up duration,” he said. “Lastly, the additive effect of combination treatment and variable dosing was not captured in the present analysis, and further statistical analysis is required for re-epithelialization–based outcome measures and length of stay to ascertain significance.”
Mr. Mansour reported having no financial disclosures.