Department of Internal Medicine, Singapore General Hospital, Singapore.
Health Services Research Unit, Division of Medicine, Singapore General Hospital, Singapore.
JAMA Dermatol. 2020 Dec 1;156(12):1294-1299. doi: 10.1001/jamadermatol.2020.3654.
Epidermal 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.
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.
In-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.
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.
In 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.
表皮坏死松解症是一种罕见的严重皮肤药物反应,与高死亡率相关。ABCD-10 评分(年龄、碳酸氢盐、癌症、透析、10%体表面积)是一种新的预测表皮坏死松解症死亡率的预后评分,最近在美国得到了开发和验证。然而,据我们所知,它在其他队列中仍有待外部验证。
评估 ABCD-10 在当代亚洲队列中的表现,并将其与毒性表皮坏死松解症评分(SCORTEN)进行比较,并研究时间和免疫调节治疗与 ABCD-10 和 SCORTEN 表现的关系。
设计、设置和参与者:这项回顾性队列研究在 2003 年 1 月至 2019 年 3 月期间进行了 17 年,纳入了 196 名来自新加坡总医院的表皮坏死松解症患者,新加坡总医院是表皮坏死松解症的国家转诊中心。
住院死亡率。使用接受者操作特征曲线下面积和校准图分别评估和比较每个风险评分的区分度和校准度。
在 196 名患者(中位数[四分位间距]年龄,56[42-70]岁;116 名女性[59.2%])中,45 名(23.0%)未存活出院。ABCD-10 中的所有危险因素均与住院死亡率显著相关。然而,入院前的透析,ABCD-10 中权重最大的因素,在本队列中表现较弱(比值比,3.7;95%CI,1.0-13.2,P=.04)。尽管 ABCD-10 和 SCORTEN 的区分度没有差异(曲线下面积:ABCD-10,0.78;95%CI,0.72-0.85;与 SCORTEN,0.77;95%CI,0.69-0.84;P=.53),但 ABCD-10 的校准度较差。从校准图的图形分析来看,ABCD-10 在较低的评分范围内低估了死亡率,在较高的评分范围内高估了死亡率。相比之下,SCORTEN 通常校准良好,尽管在较高的评分范围内可能高估了死亡率。对校准图的评估表明,与仅接受支持性治疗的患者相比,SCORTEN 在后期或使用免疫调节治疗时,死亡率的高估程度逐渐增加。即使在后期或接受免疫调节治疗的患者中,ABCD-10 的校准仍很差。
在本队列中,SCORTEN 在预测表皮坏死松解症的死亡率方面优于 ABCD-10。然而,它确实显示出与时间相关的校准恶化,导致对死亡率风险的高估。未来的研究可能需要考虑修订现有的 SCORTEN,因为它目前具有良好的区分度。