Stevens-Johnson Syndrome/Toxic Epidural Necrolysis 2017: Building Multidisciplinary Networks to Drive Science and Translation

项目摘要 Stevens-Johnson综合征/中毒性表皮坏死松解症（SJS/TEN）是一种免疫介导的疾病， 药物引起的艾滋病毒/艾滋病罕见（估计发生率为1-3/1，000，000），但集体负担很高。它是最古老的 皮肤药物不良反应，通常死亡率>30%，短期和长期发病率显著。 在美国和国际上，SJS/TEN研究、预防和临床护理的进展需要 多个学科的协调，如重症监护，外科，皮肤科，眼科，临床 药理学，流行病学，监管科学，信息学，免疫学，遗传学，皮肤生物学和其他领域的 基础科学通过国家卫生研究院组织并由国家人类基因组研究所领导的为期两天的研讨会取得了重大进展 2015年3月与FDA合作，30名已成立的SJS/TEN研究人员参与其中，以确定优先事项。有 目前没有正在进行的专门针对SJS/TEN的应急小组或会议，以允许成立多学科小组 科学家和临床医生参加一次会议。该提案寻求支持“史蒂文斯-约翰逊 综合征/中毒性表皮坏死松解症2017：建立多学科网络，推动科学与翻译”， 满足这一未满足的需求。会议将于2017年3月2日在美国科学院 皮肤病学会议（2017年3月3日至6日），佛罗里达奥兰多。该计划将吸引约200名与会者， 将鼓励新的研究人员，学员，妇女和少数民族跨多个科学和临床学科， 患者和患者倡导团体。为期一天的计划将包括介绍和参与更多的 20多名国内外专家，无论是在SJS/TEN领域还是在科学学科领域， 为SJS/TEN的发展做出贡献。该计划将包括各种演示格式，包括主题演讲 专题介绍、圆桌讨论和海报展示会。一个组织委员会已经成立， 杰出的国家和国际代表，包括妇女、少数民族、新调查员、一名受训人员和 SJS基金会成员本次会议将有助于1）创建网络; 2）优先考虑研究目标和未回答的问题 问题; 3）为新的研究人员和受训人员提供指导; 4）召集主要研究人员， 尖端研究; 5）解决SJS/TEN研究短期和长期进展的潜在障碍。是 预计这次会议的一个重要成果将是建立一个协同的全球研究人员网络 建立持久和富有成效的合作关系。会议记录将分发给 通过网络录音和同行评审的出版物。与NIAMS和NIH的使命一致， 会议将成功地聚集关键的研究人员建立或做相关的工作，这严重危及生命 皮肤药物不良反应的传播，教育，翻译和燃料的重大进展， SJS/TEN的病因、治疗和预防将大大促进人类健康。

Project Summary Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) an immunologically-mediated disease that is usually drug-induced, is rare (estimated incidence 1-3/1,000,000) but has a high collective burden. It is the severest of cutaneous adverse drugs reactions, often with >30% mortality and significant short and long-term morbidity. Advancement in SJS/TEN research, prevention and clinical care within the United States and internationally requires considerable coordination of multiple disciplines such as critical care, surgery, dermatology, ophthalmology,clinical pharmacology, epidemiology, regulatory science, informatics, immunology, genetics, skin biology and other areas of basic science. Significant progress was achieved through a two-day workshop organized by the NIH and led by the NHGRI in collaboration with the FDA in March 2015 which involved 30 established SJS/TEN researchers to set priorities. There is currently no ongoing contingency group or conference dedicated to SJS/TEN that would allow a multidisciplinary group of scientists and clinicians to engage at a single meeting. This proposal seeks support for “Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis 2017: Building Multidisciplinary Networks to Drive Science & Translation” to address this unmet need. The meeting will take place March 2, 2017 immediately preceding the American Academy of Dermatology meeting (March 3-6, 2017) in Orlando, Florida. The program will attract approximately 200 attendees and will encourage new investigators, trainees, women and minorities across multiple scientific and clinical disciplines, patients and patient advocacy groups. The one-day program will include presentations and involvement from more than 20 domestic and international experts both within the area of SJS/TEN or in scientific disciplines that have much to contribute to the advancement of SJS/TEN. The program will include a variety of presentation formats including keynote presentations, round table discussions and a poster session. An organizing committee has been assembled that has outstanding national and international representation and includes women, minorities, new investigators, a trainee and an SJS foundation member. This meeting will serve to 1) Create networks; 2) Prioritize research goals and unanswered questions; 3) Provide mentorship for new investigators and trainees; 4) Assemble key researchers for presentation of cutting edge research; and 5) Address potential barriers to short and long-term progress in SJS/TEN research. It is anticipated that an important outcome of this meeting will be the creation of a synergistic global network of researchers that will have a durable and productive collaborative relationship. The proceedings of this meeting will be disseminated through web-recording and a peer-reviewed publication. Consistent with the mission of the NIAMS and NIH this meeting will successfully assemble key researchers established or doing work of relevance to this severe life-threatening cutaneous adverse drug reaction for dissemination, education, translation and fuel significant progress in defining causes, treatment and prevention of SJS/TEN that will significantly advance human health.