SJS-TEN Overlap syndrome

  • 文章类型: Journal Article
    史蒂文斯-约翰逊综合征(SJS)和毒性表皮坏死松解症(TEN)存在于一系列引起表皮脱离和角质形成细胞坏死的自身免疫疾病中。由于这些情况的罕见发生,治疗算法存在巨大的异质性。为了更好地理解药物免疫抑制疗法对生存的影响,作者查询了一个多机构数据网络。本研究的数据来自TriNetX研究网络,包含来自国际医疗保健组织联盟的ICD-9/ICD-10编码数据的平台。查询了71个机构,以识别诊断为SJS的成年患者,十或SJS-TEN重叠。根据接受的治疗创建队列:全身性类固醇(SS),苯海拉明(DH),环孢菌素(CS),静脉注射免疫球蛋白(IVIG),肿瘤坏死因子α抑制剂(TNFαi),或治疗的组合。然后将队列与接受支持性护理的患者进行倾向匹配。仅接受上述治疗之一的患者90天死亡率没有显着降低。与支持治疗相比,接受CS或IVIG作为多种治疗的患者死亡风险显著增加(CS:RR=1.583,95%CI[1.119,2.240];IVIG:RR=2.132,95%CI[1.485,3.059])。尽管他们经常使用,这项研究的分析提示,这些治疗方法均不比单独的支持治疗具有显著的90天死亡率生存率.这些结果突出了治疗的异质性,并强调了对SJS和TEN结果进行关键前瞻性评估的必要性。
    Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) exist on a spectrum of autoimmune conditions which cause epidermal detachment and keratinocyte necrosis. Due to the rare incidence of these conditions, a dramatic heterogeneity in treatment algorithms exists. To better appreciate pharmacologic immunosuppressive therapies\' impact on survival, the authors queried a multi-institutional data network. Data for this study was extracted from TriNetX Research Network, a platform that contains ICD-9/ICD-10 coding data from a consortium of international healthcare organizations. Seventy-one institutions were queried to identify adult patients diagnosed with SJS, TEN or SJS-TEN Overlap. Cohorts were created based on the therapy received: systemic steroids (SS), diphenhydramine (DH), cyclosporine (CS), intravenous immunoglobulin (IVIG), tumor necrosis factor alpha inhibitors (TNFαi), or a combination of treatments. Cohorts were then propensity matched with patients who received supportive care. Patients who only received one of the above treatments showed no significant reduction in 90-day mortality. Patients who received CS or IVIG as part of their multitherapy showed a significantly increased risk of death when compared to supportive care (CS: RR = 1.583, 95% CI [1.119, 2.240]; IVIG: RR = 2.132, 95% CI [1.485, 3.059]). Despite their frequent utilization, this study\'s analysis suggests that none of these therapies confer significant 90-day mortality survival over supportive care alone. These results highlight the heterogeneity of therapies and emphasize the need for critical prospective appraisal of their outcomes in SJS and TEN.
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  • 文章类型: Journal Article
    背景:涉及体表面积(BSA)≥10%的表皮坏死松解症(EN)通常会并发细菌感染。
    目的:我们试图描述涉及BSA≥10%的EN中血流感染(BSIs)的流行病学以及皮肤培养物的诊断性能,以预测从BSIs中分离出的病原体。
    方法:这项回顾性单中心观察性研究于2009年至2017年进行。包括所有在急性期转诊的涉及BSA≥10%的EN患者。收集所有临床和细菌学相关数据(血液和皮肤培养结果,number,以及BSI的严重程度和时间)。灵敏度,特异性,并研究了皮肤培养物的预测值和细菌接种物的影响。
    结果:在98名患者中,46例(46.9%)在住院期间发生≥1次BSI(BSI由金黄色葡萄球菌[n=17,36.9%]和铜绿假单胞菌[n=17,36.9%]引起)。32例(71.1%)皮肤培养与血培养结果一致。金黄色葡萄球菌的阳性预测值和阴性预测值分别为57.7%和89.4%,铜绿假单胞菌的阳性预测值分别为50.0%和80.9%。分别。BSI随金黄色葡萄球菌皮肤接种物的增加而增加。
    结论:这是一个回顾性的单中心设计,BSI总数较低。
    结论:金黄色葡萄球菌和铜绿假单胞菌的皮肤培养可能有助于预测BSIs涉及的病原体。
    BACKGROUND: Epidermal necrolysis (EN) involving ≥10% of the body surface area (BSA) is often complicated by bacterial infections.
    OBJECTIVE: We sought to describe the epidemiology of bloodstream infections (BSIs) in EN involving a BSA ≥10% and the diagnostic performances of skin cultures for predicting the pathogen(s) isolated from BSIs.
    METHODS: This retrospective single-center observational study was conducted between 2009 and 2017. All patients referred at the acute phase for EN involving a BSA ≥10% were included. All clinical and bacteriologically relevant data were collected (blood and skin cultures results, number, and severity and time of BSI). Sensitivity, specificity, and predictive values of skin cultures and impact of the bacterial inoculum were investigated.
    RESULTS: Of 98 patients, 46 (46.9%) had ≥1 BSI episode during the hospital stay (BSIs were caused by Staphylococcus aureus [n = 17, 36.9%] and Pseudomonas aeruginosa [n = 17, 36.9%]). Skin cultures were concordant with blood cultures in 32 cases (71.1%). The positive and negative predictive values were 57.7% and 89.4% for S aureus and 50.0% and 80.9% for P aeruginosa, respectively. BSI increased with cutaneous inoculum of S aureus.
    CONCLUSIONS: This was a retrospective single-center design with a low total number of BSIs.
    CONCLUSIONS: Skin cultures for S aureus and P aeruginosa may help predict the pathogens involved in BSIs.
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