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Cohort study of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis in China: evaluation of risk models and new predictor of pulmonary consolidation on computed tomography

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Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe diseases. This study aimed to validate the predictive ability of risk models in patients with SJS/TEN and propose possible refinement in China. Patients in the Department of Dermatology of Huashan Hospital from January 2008 to January 2019 were included. Results showed that the severity-of-illness score for TEN (SCORTEN) had a good discrimination (area under the receiver operating characteristic curve (AUC), 0.78), and it was superior to auxiliary score (AS) and ABCD-10, which indicates age, bicarbonate level, cancer, dialysis, and 10% involved body surface area (AUC, 0.69 and 0.68, respectively). The calibration of SCORTEN (Hosmer-Lemeshow goodness-of-fit test, P = 0.69) was also better than that of AS (P = 0.25) and ABCD-10 (P = 0.55). SCORTEN and ABCD-10 were similar (Brier score (BS), 0.04 and 0.04) in terms of accuracy of predictions. In addition, the imaging appearance of pulmonary consolidation on computed tomography was associated with high mortality. Refined models were formed using the variables and this imaging appearance. The refined AS and ABCD-10 models were similar in discrimination compared with the original SCORTEN (0.74 vs. 0.78, P = 0.23; 0.74 vs. 0.78, P = 0.30, respectively). Therefore, SCORTEN showed good discrimination performance, calibration, and accuracy, and refined AS or ABCD-10 model may be an option when SCORTEN variables are not available.

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References

  1. Schneck J, Fagot JP, Sekula P, Sassolas B, Roujeau JC, Mockenhaupt M. 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. J Am Acad Dermatol 2008; 58(1): 33–40

    Article  Google Scholar 

  2. Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol 1993; 129(1): 92–96

    Article  CAS  Google Scholar 

  3. Diphoorn J, Cazzaniga S, Gamba C, Schroeder J, Citterio A, Rivolta AL, Vighi GD, Naldi L, REACT-Lombardia study group. Incidence, causative factors and mortality rates of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in northern Italy: data from the REACT registry. Pharmacoepidemiol Drug Saf 2016; 25 (2): 196–203

    Article  CAS  Google Scholar 

  4. Hsu DY, Brieva J, Silverberg NB, Silverberg JI. Morbidity and mortality of Stevens-Johnson syndrome and toxic epidermal necrolysis in United States adults. J Invest Dermatol 2016; 136 (7): 1387–1397

    Article  CAS  Google Scholar 

  5. Bastuji-Garin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz J, Wolkenstein P. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol 2000; 115(2): 149–153

    Article  CAS  Google Scholar 

  6. Micheletti RG, Chiesa-Fuxench Z, Noe MH, Stephen S, Aleshin M, Agarwal A, Boggs J, Cardones AR, Chen JK, Cotliar J, Davis MDP, Dominguez A, Fox LP, Gordon S, Hamrick R, Ho B, Hughey LC, Jones LM, Kaffenberger BH, Kindley K, Kroshinsky D, Kwong BY, Miller DD, Mostaghimi A, Musiek A, Ortega-Loayza AG, Patel R, Posligua A, Rani M, Saluja S, Sharon VR, Shinkai K, John JS, Strickland N, Summers EM, Sun N, Wanat KA, Wetter DA, Worswick S, Yang C, Margolis DJ, Gelfand JM, Rosenbach M. Stevens-Johnson syndrome/toxic epidermal necrolysis: a multicenter retrospective study of 377 adult patients from the United States. J Invest Dermatol 2018; 138(11): 2315–2321

    Article  CAS  Google Scholar 

  7. Sekula P, Liss Y, Davidovici B, Dunant A, Roujeau JC, Kardaun S, Naldi L, Schumacher M, Mockenhaupt M. Evaluation of SCORTEN on a cohort of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis included in the RegiSCAR study. J Burn Care Res 2011; 32(2): 237–245

    Article  Google Scholar 

  8. Wang L, Mei XL. Retrospective analysis of Stevens-Johnson syndrome and toxic epidermal necrolysis in 88 Chinese patients. Chin Med J (Engl) 2017; 130(9): 1062–1068

    Article  Google Scholar 

  9. Mahar PD, Wasiak J, Cleland H, Watters D, Paul E, Marsh P, Gin D. Mortality and use of the auxiliary score in extensive toxic epidermal necrolysis patients admitted to an adult burns referral centre. Dermatology 2013; 227(2): 180–185

    Article  Google Scholar 

  10. Noe MH, Rosenbach M, Hubbard RA, Mostaghimi A, Cardones AR, Chen JK, Cotliar J, Davis MDP, Dominguez A, Fox LP, Hughey LC, Kaffenberger BH, Kroshinsky D, Kwong BY, Miller DD, Musiek A, Ortega-Loayza AG, Sharon VR, Shinkai K, Summers EM, Wanat KA, Wetter DA, Worswick S, Margolis DJ, Gelfand JM, Micheletti RG. Development and validation of a risk prediction model for in-hospital mortality among patients with Stevens-Johnson syndrome/toxic epidermal necrolysis-ABCD-10. JAMA Dermatol 2019; 155(4): 448–454

    Article  Google Scholar 

  11. Yang MS, Lee JY, Kim J, Kim GW, Kim BK, Kim JY, Park HW, Cho SH, Min KU, Kang HR. Incidence of Stevens-Johnson syndrome and toxic epidermal necrolysis: a nationwide population-based study using national health insurance database in Korea. PLoS One 2016; 11(11): e0165933

    Article  Google Scholar 

  12. Vaishampayan SS, Das AL, Verma R. SCORTEN: does it need modification? Indian J Dermatol Venereol Leprol 2008; 74(1): 35–37

    Article  CAS  Google Scholar 

  13. Firoz BF, Henning JS, Zarzabal LA, Pollock BH. Toxic epidermal necrolysis: five years of treatment experience from a burn unit. J Am Acad Dermatol 2012; 67(4): 630–635

    Article  Google Scholar 

  14. Weinand C, Xu W, Perbix W, Lefering R, Maegele M, Rathert M, Spilker G. 27 years of a single burn centre experience with Stevens-Johnson syndrome and toxic epidermal necrolysis: analysis of mortality risk for causative agents. Burns 2013; 39(7): 1449–1455

    Article  CAS  Google Scholar 

  15. Hu CH, Chang NJ, Liu EKW, Chuang SS, Chung WH, Yang JY. SCORTEN and impaired renal function related to mortality of toxic epidermal necrolysis syndrome patients in the Asian population. J Eur Acad Dermatol Venereol 2013; 27(5): 628–633

    Article  Google Scholar 

  16. Xu N, Chen X, Wu S, Ye M, Zhang M, Tong L, Zhu X, Liu J, Hu F, Bai C. Chronic pulmonary complications associated with toxic epidermal necrolysis: a case report and literature review. Exp Ther Med 2018; 16(3): 2027–2031

    PubMed  PubMed Central  Google Scholar 

  17. Hanley JA, McNeil BJ. The meaning and use of the area under a receiver operating characteristic (ROC) curve. Radiology 1982; 143 (1): 29–36

    Article  CAS  Google Scholar 

  18. Lemeshow S, Hosmer DW Jr. A review of goodness of fit statistics for use in the development of logistic regression models. Am J Epidemiol 1982; 115(1): 92–106

    Article  CAS  Google Scholar 

  19. Gerds TA, Cai T, Schumacher M. The performance of risk prediction models. Biom J 2008; 50(4): 457–479

    Article  Google Scholar 

  20. Austin PC, Tu JV. Bootstrap methods for developing predictive models. Am Stat 2004; 58(2): 131–137

    Article  Google Scholar 

  21. Yang L, Shou Y, Li F, Zhu X, Yang Y, Xu J. Retrospective study of 213 cases of Stevens-Johnson syndrome and toxic epidermal necrolysis from China. Burns 2020; 46(4): 959–969

    Article  Google Scholar 

  22. Walker CM, Abbott GF, Greene RE, Shepard JA, Vummidi D, Digumarthy SR. Imaging pulmonary infection: classic signs and patterns. AJR Am J Roentgenol 2014; 202(3): 479–492

    Article  Google Scholar 

  23. Sekula P, Dunant A, Mockenhaupt M, Naldi L, Bouwes Bavinck JN, Halevy S, Kardaun S, Sidoroff A, Liss Y, Schumacher M, Jean-Claude Roujeau for the RegiSCAR study group. Comprehensive survival analysis of a cohort of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. J Invest Dermatol 2013; 133(5): 1197–1204

    Article  CAS  Google Scholar 

  24. Cartotto R, Mayich M, Nickerson D, Gomez M. SCORTEN accurately predicts mortality among toxic epidermal necrolysis patients treated in a burn center. J Burn Care Res 2008; 29(1): 141–146

    Article  Google Scholar 

  25. Lipový B, Holoubek J, Hanslianová M, Cvanová M, Klein L, Grossová I, Zajíček R, Bukovčan P, Koller J, Baran M, Lengyel P, Eimer L, Jandová M, Košt’ál M, Brychta P, CELESTE Study Group. Toxic epidermal necrolysis data from the CELESTE multinational registry. Part II: specific systemic and local risk factors for the development of infectious complications. Burns 2018; 44(6): 1561–1572

    Article  Google Scholar 

  26. Imahara SD, Holmes JH 4th, Heimbach DM, Engrav LE, Honari S, Klein MB, Gibran NS. SCORTEN overestimates mortality in the setting of a standardized treatment protocol. J Burn Care Res 2006; 27(3): 270–275

    Article  Google Scholar 

  27. Diao M, Thapa C, Ran X, Ran Y, Lv X. A retrospective analysis of infections and antibiotic treatment in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. J Dermatolog Treat 2020; 31(1): 61–65

    Article  CAS  Google Scholar 

  28. de Prost N, Mekontso-Dessap A, Valeyrie-Allanore L, Van Nhieu JT, Duong TA, Chosidow O, Wolkenstein P, Brun-Buisson C, Maître B. Acute respiratory failure in patients with toxic epidermal necrolysis: clinical features and factors associated with mechanical ventilation. Crit Care Med 2014; 42(1): 118–128

    Article  Google Scholar 

  29. Guégan S, Bastuji-Garin S, Poszepczynska-Guigné E, Roujeau JC, Revuz J. Performance of the SCORTEN during the first five days of hospitalization to predict the prognosis of epidermal necrolysis. J Invest Dermatol 2006; 126(2): 272–276

    Article  Google Scholar 

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Acknowledgements

This research was supported by a grant from the National Natural Science Foundation of China (No. 81673060). We thank the Department of Dermatology of Huashan Hospital for offering clinical data.

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Correspondence to Yongsheng Yang or Jinhua Xu.

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Yanhong Shou, Lu Yang, Yongsheng Yang, Xiaohua Zhu, Feng Li, Bo Yin, Yingyan Zheng, and Jinhua Xu declare that they have no conflict of interest. This study was approved by the Ethics Committee of Huashan Hospital (reference number 2019–289), and all procedures followed were in accordance with the ethical standards.

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Shou, Y., Yang, L., Yang, Y. et al. Cohort study of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis in China: evaluation of risk models and new predictor of pulmonary consolidation on computed tomography. Front. Med. 15, 585–593 (2021). https://doi.org/10.1007/s11684-020-0817-2

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