Tsung-Yu Tsai1, I-Hsin Huang2, Yuan-Chen Chao3, Hua Li4, Tyng-Shiuan Hsieh3, Hsiao-Han Wang5, Yu-Ting Huang6, Chun-Yuan Chen7, Ying-Chih Cheng8, Po-Hsiu Kuo4, Yu-Chen Huang9, Yu-Kang Tu10. 1. Department of Dermatology, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan; Research Center of Big Data and Meta-analysis, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan. 2. Department of Education, Taipei Medical University Hospital, Taipei, Taiwan. 3. Department of Medical Education, National Taiwan University Hospital, Taipei, Taiwan. 4. Institute of Epidemiology and Preventive Medicine, National Taiwan University College of Public Health, Taipei, Taiwan. 5. Department of Dermatology, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan; Research Center of Big Data and Meta-analysis, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan; Department of Dermatology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan. 6. Wan Fang Hospital Library, Taipei Medical University, Taipei, Taiwan. 7. Research Center of Big Data and Meta-analysis, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan; Biostatistics Center, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan. 8. Institute of Epidemiology and Preventive Medicine, National Taiwan University College of Public Health, Taipei, Taiwan; Department of Psychiatry, Taoyuan Psychiatric Centre, Ministry of Health and Welfare, Taoyuan City, Taiwan. 9. Department of Dermatology, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan; Research Center of Big Data and Meta-analysis, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan; Department of Dermatology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan. Electronic address: dhist2002@yahoo.com.tw. 10. Research Center of Big Data and Meta-analysis, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan; Institute of Epidemiology and Preventive Medicine, National Taiwan University College of Public Health, Taipei, Taiwan; Department of Dentistry, National Taiwan University Hospital, Taipei, Taiwan. Electronic address: yukangtu@ntu.edu.tw.
Abstract
BACKGROUND: Various systemic immunomodulating therapies have been used to treat toxic epidermal necrolysis (TEN), but their efficacy remains unclear. OBJECTIVE: To perform a systematic review and network meta-analysis (NMA) evaluating the effects of systemic immunomodulating therapies on mortality for Stevens-Johnson syndrome (SJS)/TEN overlap and TEN. METHODS: A literature search was performed in online databases (from inception to October 31, 2019). Outcomes were mortality rates and Score of Toxic Epidermal Necrolysis (SCORTEN)-based standardized mortality ratio (SMR). A frequentist random-effects model was adopted. RESULTS: Sixty-seven studies involving 2079 patients were included. An NMA of 10 treatments showed that none was superior to supportive care in reducing mortality rates and that thalidomide was associated with a significantly higher mortality rate (odds ratio, 11.67; 95% confidence interval [CI], 1.42-95.96). For SMR, an NMA of 11 treatment arms showed that corticosteroids and intravenous immunoglobulin combination therapy was the only treatment with significant survival benefits (SMR, 0.53; 95% CI, 0.31-0.93). LIMITATIONS: Heterogeneity and a paucity of eligible randomized controlled trials. CONCLUSIONS: Combination therapy with corticosteroids and IVIg may reduce mortality risks in patients with SJS/TEN overlap and TEN. Cyclosporine and etanercept are promising therapies, but more studies are required to provide clearer evidence.
BACKGROUND: Various systemic immunomodulating therapies have been used to treat toxic epidermal necrolysis (TEN), but their efficacy remains unclear. OBJECTIVE: To perform a systematic review and network meta-analysis (NMA) evaluating the effects of systemic immunomodulating therapies on mortality for Stevens-Johnson syndrome (SJS)/TEN overlap and TEN. METHODS: A literature search was performed in online databases (from inception to October 31, 2019). Outcomes were mortality rates and Score of Toxic Epidermal Necrolysis (SCORTEN)-based standardized mortality ratio (SMR). A frequentist random-effects model was adopted. RESULTS: Sixty-seven studies involving 2079 patients were included. An NMA of 10 treatments showed that none was superior to supportive care in reducing mortality rates and that thalidomide was associated with a significantly higher mortality rate (odds ratio, 11.67; 95% confidence interval [CI], 1.42-95.96). For SMR, an NMA of 11 treatment arms showed that corticosteroids and intravenous immunoglobulin combination therapy was the only treatment with significant survival benefits (SMR, 0.53; 95% CI, 0.31-0.93). LIMITATIONS: Heterogeneity and a paucity of eligible randomized controlled trials. CONCLUSIONS: Combination therapy with corticosteroids and IVIg may reduce mortality risks in patients with SJS/TEN overlap and TEN. Cyclosporine and etanercept are promising therapies, but more studies are required to provide clearer evidence.