Stevens Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) 2023

项目摘要 Stevens-Johnson综合征和中毒性表皮坏死松解症（SJS/TEN）是一种免疫介导的， 通常是由皮肤和粘膜表面的分离和坏死定义的药物诱导的疾病。SJS/TEN 影响1-5/百万人/年，免疫功能低下和老年人的死亡率高达50%。 SJS/TEN患者在急性环境中接受多学科护理;然而，他们的社区和慢性 后续护理往往支离破碎或根本不存在。长期残疾会影响他们的视力、精神和一般健康 健康和生活质量，并可能使他们未来的安全药物选择有限。 这些进步突出了预测、预防、早期诊断和更有针对性的机会。 SJS/TEN的治疗，这种疾病的相对罕见，需要协调 多学科研究网络。5，6特别的利益相关者包括皮肤科医生，烧伤和重症监护 专家、眼科医生、过敏症专家和免疫学家、药剂师、药理学家、心理健康专家 专家和信息学家。研究人员和SJS社区之间的这种多学科网络 幸存者及其家属是2017年，2019年举行的SJS/TEN会议取得成功的重要组成部分， 和2021年。计划召开“Stevens-Johnson综合征/中毒性表皮坏死松解症2023”会议 实际上，2023年8月26日至27日（也是SJS宣传月）将建立在我们过去的SJS/TEN的成功基础上 2017年、2019年和2021年的会议。2 -5为了确保SJS/TEN 2023的成功，我们的计划重点将 包括社区参与、以病人为中心以及科学和护理方面差距。就像2021年的会议一样， 2023年的会议将几乎举行，因为全球在疫苗和安全旅行方面的不平等。 2019冠状病毒病疫情。SJS/TEN 2023的科学内容将围绕2021年确定的优先事项 会议这些措施包括：1）预防、预测和调控; 2）早期诊断、风险分层和 确定罪魁祸首药物; 3）以证据为基础的短期护理和随访;以及4）了解 机制和促进护理创新。我们的成果将解决各种问题， 通过广泛的社区参与，在整个计划中， 社区和以病人为中心的会议。其他产品包括扩大参与者的多样性 科学和社区团体。此外，我们将建立和衡量多学科网络的增长 导致赠款资金，出版物，新调查员的职业发展，创新，实施， 与SJS社区的持续接触。本次会议符合NIH的广泛使命， 国家关节炎、肌肉骨骼和皮肤病研究所鼓励治疗、预防和 研究进展、基础和临床科学家的研究培训以及社区参与。

Project Summary Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) is an immunologically mediated and typically drug-induced disease defined by separation and necrosis of skin and mucosal surfaces. SJS/TEN affects 1-5/million persons/year, and mortality in immunocompromised and elderly adults is up to 50%.1-3 SJS/TEN patients receive multidisciplinary care in the acute setting; however, their community and chronic follow-up care is often fragmented or non-existent. Long-term disabilities affect their vision, mental and general health, and quality of life and may leave them with limited safe future drug options.2-5 Although many research advances have highlighted opportunities for prediction, prevention, earlier diagnosis, and more targeted treatments for SJS/TEN, the relative rareness of the disease has created the need for coordinated multidisciplinary research networks.5, 6 Particular stakeholders include dermatologists, burn and critical care specialists, ophthalmologists, allergists and immunologists, pharmacists, pharmacologists, mental health specialists and informaticians. Such multidisciplinary networking between researchers and a community of SJS survivors and their families has been integral to the success of previous SJS/TEN meetings held in 2017, 2019, and 2021. The meeting “Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis 2023” planned to occur virtually August 26-27, 2023 (also SJS Awareness Month), will build off the success of our past SJS/TEN meetings in 2017, 2019, and 2021.2-5 To ensure the success of SJS/TEN 2023, our program highlights will include community engagement, patient focus, and disparities in science and care. Like the 2021 meeting, the 2023 meeting will be held virtually due to ongoing global inequality in vaccines and safe travel access during the COVID-19 pandemic. The scientific content of SJS/TEN 2023 will center around priorities identified in the 2021 meeting. These include 1) Prevention, prediction, and regulation; 2) Earlier diagnosis, risk stratification and identification of the culprit drug; 3) Evidence-based short-term care and follow-up; and 4) Understanding mechanisms and promoting innovation in care. Our outcomes will address concerns across diverse constituencies of patients through broad community engagement throughout the program as well as dedicated community and patient-focused sessions. Other products include expansion of the diversity of the engaged scientific and community groups. In addition, we will build and measure the growth of multidisciplinary networks leading to grant funding, publications, career development of new investigators, innovation, implementation, and ongoing engagement with the SJS community. This meeting aligns with the broad mission of the NIH and National Institute of Arthritis and Musculoskeletal and Skin disease to encourage treatment, prevention, and research progress, research training of basic and clinical scientists, and community engagement.