BACKGROUND: Toxic epidermal necrolysis (TEN) is a severe cutaneous adverse drug reaction with a mortality of 40%. Intravenous immunoglobulin (IVIg) is widely used as a specific treatment for this reaction, although evidence of its benefit is conflicting. OBJECTIVES: We sought to evaluate whether the use of IVIg improved mortality in patients with Stevens-Johnson syndrome (SJS)/TEN overlap and TEN. METHODS: We retrospectively analysed data for 64 patients with SJS/TEN overlap and TEN who were treated with IVIg at a single referral centre. The primary outcome analysed was in-hospital mortality. Predicted mortality was calculated based on severity-of-illness score for TEN (SCORTEN) values. Secondary analyses of survival based on IVIg dosages and prior corticosteroid exposure were also performed. RESULTS: There were 28 cases of SJS/TEN overlap and 36 cases of TEN, with a mean SCORTEN value of 2·6. The mean dose of IVIg given was 2·4 g kg(-1) and the mean delay from the onset of epidermal detachment to administration of IVIg was 3·2 days. There were 20 deaths (31%) in our cohort. The standardized mortality rate was 1·10 (95% confidence interval 0·62-1·58). Subgroup analysis comparing survivors and nonsurvivors showed a higher SCORTEN in nonsurvivors (3·4 vs. 2·2). There were no differences with regard to the dosage, delay and duration of IVIg administration. When stratified according to dosage, there was no mortality difference between patients who receive high-dose (≥ 3 g kg(-1) ) vs. low-dose (< 3 g kg(-1) ) IVIg. CONCLUSIONS: This study shows that the use of IVIg does not yield survival benefits in SJS/TEN overlap and TEN, even when corrected for IVIg dosages.
BACKGROUND: Toxic epidermal necrolysis (TEN) is a severe cutaneous adverse drug reaction with a mortality of 40%. Intravenous immunoglobulin (IVIg) is widely used as a specific treatment for this reaction, although evidence of its benefit is conflicting. OBJECTIVES: We sought to evaluate whether the use of IVIg improved mortality in patients with Stevens-Johnson syndrome (SJS)/TEN overlap and TEN. METHODS: We retrospectively analysed data for 64 patients with SJS/TEN overlap and TEN who were treated with IVIg at a single referral centre. The primary outcome analysed was in-hospital mortality. Predicted mortality was calculated based on severity-of-illness score for TEN (SCORTEN) values. Secondary analyses of survival based on IVIg dosages and prior corticosteroid exposure were also performed. RESULTS: There were 28 cases of SJS/TEN overlap and 36 cases of TEN, with a mean SCORTEN value of 2·6. The mean dose of IVIg given was 2·4 g kg(-1) and the mean delay from the onset of epidermal detachment to administration of IVIg was 3·2 days. There were 20 deaths (31%) in our cohort. The standardized mortality rate was 1·10 (95% confidence interval 0·62-1·58). Subgroup analysis comparing survivors and nonsurvivors showed a higher SCORTEN in nonsurvivors (3·4 vs. 2·2). There were no differences with regard to the dosage, delay and duration of IVIg administration. When stratified according to dosage, there was no mortality difference between patients who receive high-dose (≥ 3 g kg(-1) ) vs. low-dose (< 3 g kg(-1) ) IVIg. CONCLUSIONS: This study shows that the use of IVIg does not yield survival benefits in SJS/TEN overlap and TEN, even when corrected for IVIg dosages.
Authors: Katie D White; Riichiro Abe; Michael Ardern-Jones; Thomas Beachkofsky; Charles Bouchard; Bruce Carleton; James Chodosh; Ricardo Cibotti; Robert Davis; Joshua C Denny; Roni P Dodiuk-Gad; Elizabeth N Ergen; Jennifer L Goldman; James H Holmes; Shuen-Iu Hung; Mario E Lacouture; Rannakoe J Lehloenya; Simon Mallal; Teri A Manolio; Robert G Micheletti; Caroline M Mitchell; Maja Mockenhaupt; David A Ostrov; Rebecca Pavlos; Munir Pirmohamed; Elena Pope; Alec Redwood; Misha Rosenbach; Michael D Rosenblum; Jean-Claude Roujeau; Arturo P Saavedra; Hajirah N Saeed; Jeffery P Struewing; Hirohiko Sueki; Chonlaphat Sukasem; Cynthia Sung; Jason A Trubiano; Jessica Weintraub; Lisa M Wheatley; Kristina B Williams; Brandon Worley; Wen-Hung Chung; Neil H Shear; Elizabeth J Phillips Journal: J Allergy Clin Immunol Pract Date: 2018 Jan - Feb