A Review of the Systemic Treatment of Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis
Abstract
:1. Introduction
2. Pathogenesis
3. Literature Review for Systemic Treatment for SJS/TEN
4. Management
4.1. Culprit Drugs Identification and Withdrawal
4.2. Severity-of-Illness Score for TEN (SCORTEN)
4.3. Supportive Therapy
4.4. Systemic Corticosteroids
4.5. Intravenous Immunoglobulin (IVIg)
4.6. Combination of Systemic Corticosteroids and IVIg
4.7. Cyclosporine A (CsA)
4.8. TNF-Alpha (TNF-α) Inhibitors
4.9. Combination of Biologic Anti-TNF-α and Corticosteroids
4.10. Combination of Biologic Anti-TNF-α with Other Treatments
4.11. Plasmapheresis
5. Limitations
6. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Authors | Included Studies/Treatment | Summary of Potential Treatments with Benefits |
---|---|---|
Huang et al., 2012 [16] | 17 studies/IVIg | Both high-dose and low-dose IVIg were not associated with survival benefit. |
Barron et al., 2015 [17] | 13 studies/IVIg | Increasing dose of IVIg was associated with decreased mortality. |
Ye et al., 2016 [18] | 26 studies/IVIg + corticosteroid | Combination of IVIg and corticosteroid markedly reduced recovery time but not mortality. |
Huang et al., 2016 [19] | 11 studies/IVIg | IVIg was ineffective in reducing mortality in TEN patients, even at high-dose. |
Zimmermann et al., 2017 [20] | 96 studies/multiple | Glucocorticoids and cyclosporine were the most promising treatment. |
Ng et al., 2018 [21] | 9 studies/cyclosporine | Cyclosporine significantly reduced mortality. |
Zhang et al., 2019 [22] | 27 studies/TNF-α inhibitors | Biologic TNF-α inhibitors (infliximab and etanercept) are safe and effective treatments. |
Patel et al., 2021 [23] | 24 studies/multiple | Cyclosporine reduced mortality in TEN patients. Etanercept and combination of IVIg and corticosteroid and were also promising. |
Torres-Navarro et al., 2021 [24] | 38 studies/multiple | The meta-regression analysis confirmed that cyclosporine and combination of IVIg and corticosteroid were associated with less deaths than predicted by SCORTEN. |
Sachdeva et al., 2021 [25] | 38 studies/biologics | TNF-α inhibitors monotherapy improved outcomes and may be safer compared to combination therapy. |
Tsai et al., 2021 [26] | 66 studies/multiple | Combination of IVIg and corticosteroid was the only treatment with significant survival benefits. |
Krajewski et al., 2022 [27] | 42 studies/multiple | The lowest mortality was found in etanercept group followed by cyclosporine. |
Houschyar et al., 2021 [28] | 16 studies/multiple | Systemic glucocorticoids showed a survival benefit. Cyclosporine also showed promising results. |
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Chang, H.-C.; Wang, T.-J.; Lin, M.-H.; Chen, T.-J. A Review of the Systemic Treatment of Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis. Biomedicines 2022, 10, 2105. https://doi.org/10.3390/biomedicines10092105
Chang H-C, Wang T-J, Lin M-H, Chen T-J. A Review of the Systemic Treatment of Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis. Biomedicines. 2022; 10(9):2105. https://doi.org/10.3390/biomedicines10092105
Chicago/Turabian StyleChang, Hua-Ching, Tsung-Jen Wang, Ming-Hsiu Lin, and Ting-Jui Chen. 2022. "A Review of the Systemic Treatment of Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis" Biomedicines 10, no. 9: 2105. https://doi.org/10.3390/biomedicines10092105