Vanderbilt University Medical Center, Nashville, Tenn.
Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
J Allergy Clin Immunol Pract. 2018 Jan-Feb;6(1):38-69. doi: 10.1016/j.jaip.2017.11.023.
Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a life-threatening, immunologically mediated, and usually drug-induced disease with a high burden to individuals, their families, and society with an annual incidence of 1 to 5 per 1,000,000. To effect significant reduction in short- and long-term morbidity and mortality, and advance clinical care and research, coordination of multiple medical, surgical, behavioral, and basic scientific disciplines is required. On March 2, 2017, an investigator-driven meeting was held immediately before the American Academy of Dermatology Annual meeting for the central purpose of assembling, for the first time in the United States, clinicians and scientists from multiple disciplines involved in SJS/TEN clinical care and basic science research. As a product of this meeting, this article summarizes the current state of knowledge and expert opinion related to SJS/TEN covering a broad spectrum of topics including epidemiology and pharmacogenomic networks; clinical management and complications; special populations such as pediatrics, the elderly, and pregnant women; regulatory issues and the electronic health record; new agents that cause SJS/TEN; pharmacogenomics and immunopathogenesis; and the patient perspective. Goals include the maintenance of a durable and productive multidisciplinary network that will significantly further scientific progress and translation into prevention, early diagnosis, and management of SJS/TEN.
史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症(SJS/TEN)是一种危及生命的免疫介导的疾病,通常由药物引起,对个人、家庭和社会造成沉重负担,其年发病率为每 100 万人中有 1 至 5 例。为了显著降低短期和长期发病率和死亡率,并推进临床护理和研究,需要协调多个医学、外科、行为和基础科学学科。2017 年 3 月 2 日,在皮肤科学会年会之前,举行了一次由调查员驱动的会议,其核心目的是首次在美国召集来自多个学科的临床医生和科学家,参与 SJS/TEN 的临床护理和基础科学研究。作为这次会议的成果,本文总结了与 SJS/TEN 相关的当前知识状况和专家意见,涵盖了广泛的主题,包括流行病学和药物基因组网络;临床管理和并发症;儿科、老年和孕妇等特殊人群;监管问题和电子健康记录;导致 SJS/TEN 的新药物;药物基因组学和免疫发病机制;以及患者视角。目标包括维持一个持久而富有成效的多学科网络,这将极大地促进科学进步,并转化为 SJS/TEN 的预防、早期诊断和管理。