Assessment and Comparison of Performance of ABCD-10 and SCORTEN in Prognostication of Epidermal Necrolysis

Abstract

IMPORTANCEEpidermal necrolysis is a rare severe cutaneous drug reaction associated with high mortality. The ABCD-10 score (age, bicarbonate, cancer, dialysis, 10% body surface area), a new prognostic score for mortality in epidermal necrolysis, was recently developed and validated in the US. However, to our knowledge, it remains to be externally validated in other cohorts.OBJECTIVE To assess ABCD-10 among patients in a contemporary Asian cohort and compare its performance with the Score of Toxic Epidermal Necrosis (SCORTEN) and study the associations of time and immunomodulatory therapy with the performance of ABCD-10 and SCORTEN.DESIGN, SETTING, AND PARTICIPANTS This retrospective cohort study was conducted over a 17-year period from January 2003 to March 2019 and included 196 patients with epidermal necrolysis who were recruited from Singapore General Hospital, the national referral center for epidermal necrolysis. MAIN OUTCOMES AND MEASURESIn-hospital mortality. Discrimination and calibration of each risk score were assessed and compared using the area under the receiver operating characteristic curve and calibration plot, respectively. RESULTS Among 196 patients (median [interquartile range] age, 56 [42-70] years; 116 women [59.2%]), 45 (23.0%) did not survive to discharge. All risk factors in ABCD-10 were significantly associated with in-hospital mortality. However, dialysis before admission, the most heavily weighted factor in ABCD-10, performed weaker in this cohort (odds ratio, 3.7; 95% CI, 1.0-13.2, P = .04). Although the discrimination of ABCD-10 and SCORTEN did not differ (area under the curve: ABCD-10, 0.78; 95% CI, 0.72-0.85; vs SCORTEN, 0.77; 95% CI, 0.69-0.84; P = .53), the calibration of ABCD-10 was poorer compared with SCORTEN. From graphical analysis of the calibration plots, ABCD-10 showed mortality underestimation at lower score ranges and overestimation at higher score ranges. By contrast, SCORTEN was generally well calibrated, although at higher score ranges mortality may be overestimated. Assessment of calibration plots showed that there was increasing overestimation of mortality by SCORTEN during the later period or when immunomodulatory therapy was used compared with patients treated with supportive care alone. Calibration of ABCD-10 remained poor even during the later period or among patients treated with immunomodulatory therapy. CONCLUSIONS AND RELEVANCEIn this cohort of patients, the performance of SCORTEN was superior to ABCD-10 in mortality prognostication in epidermal necrolysis. However, it did display time-associated deterioration in calibration leading to overestimation of mortality risk. Future studies may consider revising the existing SCORTEN given its current good discrimination.

Accuracy of SCORTEN in predicting mortality

“…We use a combination of plasmaphereses and intravenous immunoglobulins. Our observation is not the rst to show the observed decrease in mortality risk in a group of patients treated with immunomodulation [20]. Differences between predicted and actual mortality, as well as a tendency for SCORTEN to overestimate the risk, were also observed by Imahara et.al [21].…”

“…Some authors use SCORTEN not only to predict mortality, but also to evaluate the e cacy of immunomodulatory treatment [18,19] or supportive therapy evaluation. However, there is evidence suggesting that SCORTEN tends to overestimate mortality risk in certain populations [20].…”

Neutrophil : lymphocyte ratio is associated with disease severity and mortality in patients with Stevens–Johnson syndrome/toxic epidermal necrolysis

“…1 Recently, a new SJS/TEN mortality risk-prediction model, the ABCD-10 (age, bicarbonate, cancer, dialysis, 10% body surface area), has been developed with promising results 2 but may be inferior to SCORTEN. 3 As we know, systemic inflammatory response is an important adverse factor in the development of many diseases. Some traditional inflammatory factors, like C-reactive protein (CRP) and procalcitonin (PCT), are considered as indicators for the severity of inflammation.…”

Combined use of cyclosporine in the treatment of Stevens–Johnson syndrome/toxic epidermal necrolysis

“…Some previous studies compared these two models on the prognosis of SJS/TEN, all suggesting a better performance of SCORTEN than ABCD‐10 in the prognosis of TEN. Even in side‐effects prediction, SCORTEN performed better than ABCD‐10 36–38 . Consequently, the status of SCORTEN in the SJS/TEN prognosis remains irreplaceable until a more appropriate model is defined.…”