SCORTEN和ABCD-10评估Stevens-Johnson综合征/中毒性表皮坏死松解症预后的比较

【摘要】 目的 比较中毒性表皮坏死松解症（TEN）疾病严重程度评分（SCORTEN）及ABCD-10评分系统对Stevens-Johnson综合征（SJS）/TEN患者出现死亡结局的预测评估效能。方法 对2010年1月至2021年4月于四川省人民医院住院治疗的85例SJS/TEN患者的临床数据进行回顾性分析。比较SCORTEN及ABCD-10每个分数层患者的预期死亡率及实际死亡率,采用受试者操作特征（ROC）曲线及Hosmer-Lemeshow拟合优度检验评估SCORTEN及ABCD-10对死亡的预测力及校准度。结果 85例患者中男37例,女48例,年龄14 ~ 88（52.36 ± 19.31）岁,其中SJS 61例,SJS/TEN重叠6例,TEN 18例。死亡10例,病死率为11.76%。在SCORTEN和ABCD-10的危险因素中,年龄 > 40岁或 ≥ 50岁、入院第1天表皮剥脱 > 10%体表面积、心率 > 120次/min、血清尿素氮 > 10 mmol/L、血清碳酸氢盐 < 20 mmol/L与该组患者死亡存在统计学相关性（χ2值分别为4.46、6.18、25.50、15.13、7.59、8.38,均P < 0.05）,而伴恶性肿瘤、血清葡萄糖水平 > 14 mmol/L及入院前透析与死亡无统计学相关性（χ2值分别为0.35、0.10、1.38,均P > 0.05）。SCORTEN和ABCD-10每个分数层患者的预期死亡率与实际死亡率差异均无统计学意义（均P > 0.05）。ROC曲线显示SCORTEN及ABCD-10均对死亡有良好的预测力（曲线下面积分别为0.874、0.867,95% CI分别为0.758 ~ 0.990、0.773 ~ 0.962）,但SCORTEN的模型拟合度优于ABCD-10（P值分别为0.944、0.048）。结论 SCORTEN及ABCD-10评分系统均可在早期较准确地预测SJS/TEN患者的死亡概率,其中以SCORTEN校准更好,预测性能更优。     

重症多形红斑型药疹52例和中毒性表皮坏死松解症31例回顾性分析

目的比较重症多形红斑(SJS)和中毒性表皮坏死松解症(TEN)的临床特征和实验室检查指标的差异。方法对2000年7月-2012年2月本院收治的83例SJS和TEN患者的临床资料进行回顾性分析,用SCORTEN评分系统对疾病的严重程度和病人的预后进行评估。结果引起SJS和TEN的药物以抗生素为主,其次是非甾体类抗炎药。TEN组的电解质异常的例数明显高于SJS组(P<0.05);肝功能损害是SJS和TEN最常见的并发症;SJS组和TEN组共有81例给予系统用糖皮质激素治疗,2例仅用人免疫球蛋白治疗,SJS组7例和TEN组15例给予激素联合人免疫球蛋白治疗。SCORTEN评分超过3分的有4例,其中1例死亡。结论抗生素是引起重症药疹最常见的药物,系统应用糖皮质激素尤其是联合人免疫球蛋白治疗可能是降低SJS和TEN死亡率的有效手段之一。     

52例重症多形红斑和14例中毒性表皮坏死松解症回顾性分析

目的:分析重症多形红斑(SJS)和中毒性表皮坏死松解症(TEN)的致病因素、临床特征、实验室检查指标以及治疗方法的差异。方法:对2013年1月-2018年1月本院收治的52例SJS和14例TEN患者的临床资料进行回顾性分析。结果:引起SJS和TEN的可疑致敏药物以抗生素为主。发热、肝功能异常和肾功能异常是SJS和TEN最常见的并发症。SJS和TEN患者共有65例系统使用糖皮质激素治疗,35例使用人免疫球蛋白(IVIG)、24例使用环孢素治疗。SCORTEN评分超过3分的有11例。共4例死亡。结论:抗生素是SJS和TEN最常见的致敏药物,肝肾损伤及发热是最常见的临床并发症。系统应用糖皮质激素联合环孢素,早期予以IVIG可能是治疗SJS和TEN的有效手段。     

Stevens-Johnson综合征和中毒性表皮坏死松解症眼部受累与SCORTEN分值相关性分析

目的 :探讨Stevens-Johnson综合征(SJS)和中毒性表皮坏死松解症(TEN)眼部受累与SJS/TEN严重度(SCORTEN)评分分值的相关性。方法:收集眼部受累的36例SJS和TEN患者,分析轻、中及重度眼部受累分布及其与SCORTEN分值的关系。结果:轻、中及重度眼部受累分别占55.5%、30.5%和16.6%,以轻度眼部受累为主,但差异无统计学意义(P=0.869);SCORTEN分值分别为(2.00±1.45)分、(2.63±1.74)分及(2.83±1.47)分,差异亦无统计学意义(P=0.393)。结论 :SJS和TEN眼黏膜受累严重程度与SCORTEN分值无关。     

静脉注射丙种球蛋白和糖皮质激素治疗重症大疱性药疹65例

目的 比较静脉注射丙种球蛋白(IVIG)联合糖皮质激素与糖皮质激素单用治疗莺症大疱性药疹的疗效.方法 从1993-2007年共收集65例重症大疱性药疹病例.使用中毒性表皮坏死松解症评分(SCORTEN)进行分析.2001年后的病例采用联合治疗,丙种球蛋白剂量0.4 g·kg-1·d-1连用5 d,此前糖皮质激素治疗的病例作为对照.结果 在45例糖皮质激素治疗的病例中10例死亡,而预期死亡数8.63.标准死亡比(SMR)分析显示接受糖皮质激素治疗患者的死亡率较常规治疗高16%(SMR=1.16;95%CI 0.56-2.13).20例接受联合治疗患者中3例死亡,而预期死亡数3.51(SMR=0.85;95%CI 0.18-2.50).在中毒性表皮坏死松解症(TEN)、Stevens-Johnson综合征(SJS)患者中两种疗法死亡率差异均无统计学意义(P>0.05).在TEN患者中,联合疗法较之糖皮质激素疗法疾病停止进展时间及总住院时间缩短(t=2.46,3.14,P值均<0.05).但糖皮质激素减量时间无差异(t=-0.045,P>0.05);SJS患者结果相同(t=2.334,t=2.275,t=1.655,P<0.05,<0.05,>0.05 o结论 IVIG和糖皮质激素联合治疗较之仅用糖皮质激素治疗显示出死亡率降低的趋势,并能早期控制病情,缩短住院时间;但联合治疗并不能使糖皮质激素早期减量.     

25例中毒性表皮坏死松解症住院患者治疗的回顾与分析

目的介绍中毒性表皮坏死松解症(TEN)这种高死亡率药疹的临床表现和治疗方法。方法分析2008年8月—2012年10月本科收入院治疗的25例TEN患者的临床特征、预后因素及治疗。结果所有病例都由药物引起。抗生素为最常见的原因,其他有抗癫痫类药物、卡马西平、别嘌呤醇和非甾体抗炎药物。败血症是最常见的并发症,其次是肝和肾功能损伤。入院后均给予糖皮质激素治疗(以强的松计算)1~1.5 mg/(kg·d),并给予静脉注射用人免疫球蛋白(IVIG)600~800 mg/(kg·d)静脉滴注4~7 d。血浆置换术联合激素治疗伴有肿瘤的2例患者。除了SCORTEN评分较高的患者死亡,其他患者均治愈。结论 TEN患者的预后与其SCORTEN评分有关。新的治疗方法对今后TEN的治疗有很大的帮助。     

血液灌流联合连续性肾脏替代疗法治疗重症药疹临床分析

目的:评价血液灌流(HP)联合连续性肾脏替代疗法(CRRT)治疗重症药疹的疗效。方法:回顾性分析2009年6月—2013年6月该院救治成功的10例重症多形红斑(SJS)及19例中毒性表皮坏死松解症(TEN)患者,根据临床治疗方案分为常规治疗组与HP+CRRT组,比较2组患者治疗前SCORTEN评分,并比较体温控制时间、皮损控制时间及住院天数。结果:2组治疗前SCORTEN评分及总预期病死率的比较差异无统计学意义(P0.05);2组体温控制时间的比较差异无统计学意义(P0.05);皮损控制时间的比较差异有统计学意义(P0.05);HP+CRRT组住院天数短于常规治疗组,差异有统计学意义(P0.05)。结论:HP联合CRRT是治疗SJS与TEN的有效手段,明显缩短病程。     

重症多形红斑与中毒性表皮坏死松解症患者的临床对比研究

目的探讨重症多形红斑(SJS)与中毒性表皮坏死松解症(TEN)的临床特点与临床治疗。方法对9例SJS和4例TEN住院患者的临床资料进行回顾性分析。结果 SJS组和TEN组患者中药物为最常见病因。TEN组的皮损范围、损害程度、粘膜病变均较SJS组更广泛、更严重。TEN患者主要表现为全身表皮松解,而SJS患者以靶形红斑为特征。TEN患者较SJS患者更易出现并发症。TEN患者病程急性期和恢复期时间均长于SJS患者。9例SJS和4例TEN患者早期均予以足量糖皮质激素治疗,TEN患者同时联用免疫球蛋白,13例患者均获痊愈。结论药物是SJS和TEN发病最主要的原因。TEN患者较SJS患者病变广泛且严重。药物诱发的SJS和TEN患者早期使用足量激素十分必要,TEN患者尚需静脉联用免疫球蛋白,提高抢救成功率。     

卡马西平致Stevens-Johnson综合征和中毒性表皮坏死松解症24例临床特点及治疗分析

目的探讨卡马西平所致Stevens-Johnson综合征(Stevens-Johnson syndrome,SJS)和中毒性表皮坏死松解症(toxic epidermal necrolysis,TEN)的临床特点及治疗转归。方法对无锡市第二人民医院皮肤科2009年—2018年住院治疗的卡马西平所致的24例SJS/TEN重症药疹临床数据进行回顾性分析。结果 24例患者中SJS患者20例,TEN患者4例。潜伏期平均(25.0±15.3)d。发热、白细胞数目异常、黏膜损伤、肝功能损伤为常见的临床症状、体征及实验室检查。24例患者均早期足量给予糖皮质激素和(或)人血丙种球蛋白治疗,1例因败血症转入内科治疗,其余23例均好转出院。结论卡马西平所致SJS/TEN重症药疹有潜伏期长、临床症状重、病程长的特点,早期足量给予糖皮质激素和(或)人血丙种球蛋白治疗是临床治愈的关键。     

单纯单次血浆置换疗法治疗17例中毒性表皮坏死松解症临床观察

目的 探讨单纯单次血浆置换（POPE）治疗中毒性表皮坏死松解症（TEN）患者的疗效及不良反应。方法 收集2010年9月至2017年12月在西安交通大学第二附属医院接受单纯单次血浆置换治疗的TEN患者,回顾性分析患者的疾病严重程度、临床疗效、住院时间和不良反应等。结果 共纳入17例患者,年龄（36.1 ± 25.4）岁,男9例,女8例,SCORTEN评分（2.1 ± 1.24）分,STENS评分治疗前为（29.9 ± 6.6）分,治疗后为（3.5 ± 1.8）分。17例患者中,单纯单次血浆置换后15例临床治愈,1例有效,1例死亡。ICU治疗时间（6.4 ± 1.8） d,总住院时间（12.1 ± 5.7） d。住院第1、4、7、10、20天时STENS评分之间差异有统计学意义（F = 18.569,P < 0.05）。除2例患者发生血浆过敏外,无其他相关显著不良反应发生。结论 单纯单次血浆置换疗法治疗TEN疗效显著,无明显不良反应。     

High-dose intravenous immunoglobulins in the treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis in Chinese patients: a retrospective study of 82 cases

Stevens-Johnson Syndrome (SJS) and Toxic epidermal necrolysis (TEN) are drug-induced diseases with a low incidence but high mortality. While there is no standard treatment, corticosteroids and intravenous immunoglobulin (IVIG) therapy have been widely used, with controversy. Our objective was to summarize the etiology and therapeutic regimen of SJS or TEN in 82 hospitalized patients in China. A retrospective study was performed on 82 patients who were diagnosed with SJS or TEN and hospitalized in Peking Union Medical College Hospital from July 1994 to August 2009. Of them, 24 were treated with IVIG plus corticosteroids (IVIG group) and the other 58 were treated with corticosteroids only (corticosteroids group). SCORTEN was used to evaluate the severity and prognosis of the patients. The efficacy of therapeutic modalities was assessed by the following parameters: starting and the maximum dose of corticosteroids, cumulative dose of corticosteroids before tapering, cumulative dose of IVIG, days of corticosteroid application before its tapering and the hospitalization days. The common agents triggering SJS/TEN in these patients were non-steroidal anti-inflammatory drugs (31 cases), anti-epileptics (18 cases), antibiotics (14 cases), antipodagrics (4 cases), sulfanilamides (4 cases) and others (11 cases), respectively. Carbamazepine was the most common drug, and induced 15 cases of SJS/TEN. The SCORTEN was significantly higher in the IVIG group than that in the corticosteroid group (2.0 ± 1.7 vs 0.8 ± 1.0, P = 0.001). Whereas no differences were observed between the two groups in the parameters including starting and maximum dose of corticosteroids, cumulative dose and the number of application days of corticosteroids before tapering and hospitalization days. However, in patients whose SCORTEN scores were 2, application of IVIG and corticosteroids shortened the duration of hospitalization from 26.4 ± 9.5 d to 18.1 ± 5.3 d (P < 0.05). No significant difference was observed in the incidence of complications between the two groups (54.2% vs 39.7%, P > 0.05). The actual mortalities were 12.5% in the IVIG group and 3.4% in corticosteroid group respectively, which were significantly lower than the predicted values (22.0% and 7.2%, respectively). Standardized mortality ratio (SMR) analysis showed a trend to a lower actual mortality (not significant) with corticosteroid treatment than the predicted mortality (SMR = 0.480; 95% CI: 0.075-1.923) and combination therapy had a tendency to reduce the mortality (not significant) rate of TEN (SMR = 0.569; 95% CI: 0.318-1.910). No significant difference in SMR was found between the two groups (P = 0.1474). Survival analysis showed that a favorable overall survival was associated with younger age (P = 0.0405). Our data indicated that early application of corticosteroids presented beneficial effects on SJS/TEN, and that combination therapy of corticosteroids and IVIG achieved a better therapeutic effect than the administration of corticosteroids alone. We recommend early treatment with IVIG at total doses of more than 2 g/kg in SJS/TEN patients whose SCORTEN are higher than 0.     

Combination therapy of intravenous immunoglobulin and corticosteroid in the treatment of toxic epidermal necrolysis and Stevens–Johnson syndrome: a retrospective comparative study in China

Background Toxic epidermal necrolysis (TEN) and Stevens–Johnson Syndrome (SJS) are drug‐induced diseases with no well‐established treatments. The application of corticosteroid therapy is controversial. Intravenous immunoglobulin (IVIG) therapy is emerging as a promising new method for the treatment of these two diseases. The efficacy of combination therapy of IVIG and corticosteroid in the treatment of TEN/SJS has seldom been reported. Methods Sixty‐five consecutive patients with either TEN or SJS, admitted over a 14‐year period from January 1993 to October 2007, were treated with corticosteroid and analyzed retrospectively using SCORTEN, a severity‐of‐illness scoring system for TEN/SJS prognosis, to evaluate efficacy. For patients admitted after January 2001, additional therapy with a dose of 0.4 g/kg/day of IVIG for 5 days was applied. Results In the 45 patients with TEN treated without IVIG, 8.63 patients were expected to die based on the SCORTEN system, but 10 deaths were observed. Standardized mortality ratio (SMR) analysis [(Σobserved deaths/Σexpected deaths) × 100] suggested that patients with TEN treated with systemic corticosteroid were 16% more likely to die than those treated with routine therapy (SMR = 1.16; 95% confidence interval, 0.56–2.13). In the further study of combination therapy, 12 patients with TEN and eight patients with SJS were admitted. There were two deaths in the TEN group and one death in the SJS group, with 3.51 deaths expected on the basis of the SCORTEN system. SMR analysis showed that combination therapy had a tendency to reduce the mortality rate of TEN (SMR = 0.85; 95% confidence interval, 0.18–2.50). Nevertheless, in both the TEN and SJS groups, the difference in mortality rate between the two therapies was not statistically significant (P = 0.651 and P = 1, respectively). In patients with TEN, combination therapy also reduced significantly the time of arrested progression (P = 0.019) and the total hospitalization time (P = 0.043), but could not reduce the time to the tapering of corticosteroid (P = 0.96). In SJS patients, the times of arrested progression and hospitalization were also reduced significantly (P = 0.019 and P = 0.0475, respectively). Likewise, the time to the tapering of corticosteroid was not reduced (P = 0.122). Conclusion Combination therapy with corticosteroid and IVIG exhibited a tendency to reduce the mortality rate in comparison with the solo administration of corticosteroid. The decrease in the mortality rate, however, was not statistically significant. Combination therapy also arrested progression earlier and decreased the hospitalization time, meaning that the total dose of corticosteroid may be reduced. Combination therapy, however, did not lead to earlier tapering of corticosteroid. No severe adverse effects of IVIG were found during treatment.     

SCORTEN评分对中毒性表皮坏死松解症和Stevens-Johnson综合征评估分析

目的 分析SCORTEN评分对中毒性表皮坏死松解症和Stevens?Johnson综合征患者预后评估的准确性。方法 回顾性分析1992年4月至2014年3月期间在北京协和医院收治的中毒性表皮坏死松解症和Stevens?Johnson综合征39例,其中死亡病例13例,按照年龄分层1∶2匹配诊断明确好转出院26例。39例患者用SCORTEN评分系统评分,计算预期死亡数,比较39例各级分层的预期死亡数与实际死亡数,绘制受试者工作特征曲线（ROC曲线）,评估SCORTEN评分的判断力。结果 39例患者中,按SCORTEN评分系统评为1分15例,2分14例,3分6例,4分4例,总预期死亡6.808例,实际死亡13例。 每个积分层预期死亡数与实际死亡数差异无统计学意义。SCORTEN评分系统的ROC曲线下面积 = 0.832 8,表明有较好的预测能力。结论 SCORTEN评分系统可在早期对中毒性表皮坏死松解症和Stevens?Johnson综合征患者死亡率作出评估。     

IVIG for the treatment of toxic epidermal necrolysis

Intravenous immunoglobulin (IVIG) has been proposed as a treatment for toxic epidermal necrolysis (TEN) and Stevens-Johnson Syndrome (SJS). A number of retrospective and prospective studies have been conducted, with varying levels of evidence for the efficacy of IVIG. Recent publications provide opposing conclusions. A multi-center, comparative, long-term analysis needs to be conducted to determine the role of IVIG in the management of patients with SJS/TEN.     

Performance of the SCORTEN in Taiwanese patients with Stevens-Johnson syndrome and toxic epidermal necrolysis

BackgroundStevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare drug-related skin conditions that are potentially life-threatening with a 20–30% mortality rate. A severity-of-illness score specified for SJS and TEN, SCORTEN, was developed in 2000. Until now, no study of SCORTEN has been conducted in Asian with SJS or TEN. The goal of the present study is to evaluate the performance of SCORTEN in predicting in-hospital mortality in Taiwanese patient with SJS or TEN.MethodsA sample of 51 patients with the diagnosis of SJS or TEN was reviewed from the chart. Performance of the SCORTEN was assessed using logistic regression.ResultsThe hospital mortality rate predicted by the SCORTEN was similar to the observed mortality rate. The performance of SCORTEN was well on each of the first 5 days of hospitalization, and best on the first day. There were two cases with active tuberculosis and five with diabetes mellitus among our eight deceased patients.ConclusionThe performance of SCORTEN is well in Taiwanese patients with SJS or TEN, especially on the first day of admission. The SCORTEN predicts a relatively accurate in-hospital mortality rate. We suggest that the SCORTEN should always be obtained during the first 24 hours, and can be repeated if the patient's condition deteriorates quickly in the first 5 days. Pre-existing conditions, such as tuberculosis and pre-existing diabetes, may need more consideration in future studies.     

Critical factors differentiating erythema multiforme majus from Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN)

Erythema multiforme majus (EMM) and Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) are severe cutaneous reactions characterised by targetoid erythematous lesions and mucocutaneous involvement. The initial skin manifestations are similar, making early diagnosis difficult. We retrospectively reviewed 36 cases of EMM and 18 cases of SJS/TEN and also evaluated 6 patients with unclassified EMM. 13 patients in the EMM group and 16 patients in the SJS/TEN group presented with a high fever (>38.5?C; p<0.001). Two or more mucous membranes were affected in 6 patients in the EMM group and 18 patients in the SJS/TEN group. Significantly more SJS/TEN than EMM patients had high levels of C-reactive protein and severe hepatic dysfunction. Thirteen EMM and 13 SJS/TEN cases were caused by medications/drugs. Skin biopsy samples showed stronger mononuclear cell infiltration in the EMM than in the SJS/TEN group (p<0.001). The mean dose of initial systemic corticosteroid used to treat EMM was lower than that used to treat SJS/TEN. No patients died in either group. Clinically, the unclassified cases mostly behaved like EMM. The results of our investigation suggest that EMM and SJS/TEN are distinct conditions and they help in differentiating these syndromes at an early stage.     

人免疫球蛋白和重组人Ⅱ型肿瘤坏死因子受体-抗体融合蛋白治疗中毒性表皮坏死松解症的对比研究

目的:评价静脉注射人免疫球蛋白（IVIG）及重组人Ⅱ型肿瘤坏死因子受体-抗体融合蛋白（rhTNFR：Fc）治疗中毒性表皮坏死松解症（TEN）的疗效。方法:收集2013—2019年武汉市第一医院使用IVIG及rhTNFR：Fc治疗的TEN患者资料。IVIG组11例,男3例,女8例,年龄25 ~ 72岁,中位TEN疾病严重...     

Dexamethasone pulse therapy for Stevens-Johnson syndrome/toxic epidermal necrolysis

Mortality in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) is high. Apart from intensive supportive therapy, no generally accepted specific treatment regimen exists. The role of corticosteroids in SJS/TEN is controversial. It is possible that high-dose pulse therapy with corticosteroids might be an improvement on long-term lower dose therapy, by combining higher efficacy with a diminished risk both of infection and of delayed wound healing. The aim of this study was to evaluate the efficacy of dexamethasone pulse therapy with respect to mortality and healing time of patients with SJS/TEN. A small, uncontrolled series of consecutive inpatients with SJS/TEN was treated with dexamethasone pulse therapy. The efficacy of this treatment was assessed retrospectively using SCORTEN. Twelve patients were included over a period of 10 years. One patient died, while SCORTEN predicted a fatal outcome of 4 patients. Stabilization was reached after 2.3 days on average, total re-epithelialization after 13.9 days. The results of this study bear no statistical relevance due to the small number of patients. In conclusion, short-term dexamethasone pulse therapy, given at an early stage of the disease, may contribute to a reduced mortality rate in SJS/TEN without increasing healing time. A larger controlled trial is warranted to investigate further the use of dexamethasone pulse therapy in SJS/TEN.