■严重的皮肤不良反应(SCAR)是一组延迟的假定T细胞介导的超敏反应,与显著的发病率和死亡率相关。尽管它们共同的全球医疗保健负担和影响,临床表型,基因组易感性,药物因果关系,治疗结果可能有所不同。我们描述了澳大利亚第一个SCAR注册中心(AUS-SCAR)的建立和结果,通过合作网络推进预防策略,SCAR的诊断和治疗。
■澳大利亚成人和青少年SCAR的前瞻性多中心登记,有计划的区域扩张。注册管理机构收集外部验证的表型数据药物因果关系,治疗和长期患者预后。此外,在参与位点收集生物贮藏标本和DNA。
■我们报告了AUS-SCAR数据库中的前100名患者。DRESS(50%)是最主要的表型,其次是SJS/TEN(39%)和AGEP(10%),年龄中位数为52岁(IQR37.5,66),男女比例为1:1。所有相关药物的中位潜伏期差异很大,但DRESS(中位15天IQR5,25)和SJS/TEN(中位21天,IQR7,27),虽然AGEP最低(中位数为2.5天,IQR1,8).与非抗生素剂(45.5%)相比,抗生素(54.5%)更常被列为主要涉及药物。SJS/TEN90天死亡率最高,为23.1%,其次是DRESS(4%)和AGEP(0%)。
■在南半球SCAR的第一个前瞻性国家表型和生物储存库中,我们证明了与其他报告的注册管理机构的显着差异;包括DRESS主导表型,不同的抗生素因果关系和低总死亡率。这项研究还强调了缺乏标准化的预防性药物基因组学措施和体外/体内诊断策略来确定药物因果关系。
■ANZCTRACTRN12619000241134。2019年2月19日注册。
UNASSIGNED: Severe cutaneous adverse reactions (SCAR) are a group of delayed presumed T-cell mediated hypersensitivities associated with significant morbidity and mortality. Despite their shared global healthcare burden and impact, the clinical phenotypes, genomic predisposition, drug causality, and treatment outcomes may vary. We describe the establishment and results from the first Australasian registry for SCAR (AUS-SCAR), that via a collaborative network advances strategies for the prevention, diagnosis and treatment of SCAR.
UNASSIGNED: Prospective multi-center registry of SCAR in Australian adult and adolescents, with planned regional expansion. The registry collects externally verified phenotypic data drug causality, therapeutics and long-term patient outcomes. In addition, biorepository specimens and DNA are collected at participating sites.
UNASSIGNED: we report on the first 100 patients enrolled in the AUS-SCAR database. DRESS (50%) is the most predominant phenotype followed by SJS/TEN (39%) and AGEP (10%), with median age of 52 years old (IQR 37.5, 66) with 1:1 male-to-female ratio. The median latency for all implicated drugs is highly variable but similar for DRESS (median 15 days IQR 5,25) and SJS/TEN (median 21 days, IQR 7,27), while lowest for AGEP (median 2.5 days, IQR 1,8). Antibiotics (54.5%) are more commonly listed as primary implicated drug compare with non-antibiotics agent (45.5%). Mortality rate at 90 days was highest in SJS/TEN at 23.1%, followed by DRESS (4%) and AGEP (0%).
UNASSIGNED: In the first prospective national phenotypic and biorepository of SCAR in the southern hemisphere we demonstrate notable differences to other reported registries; including DRESS-predominant phenotype, varied antibiotic causality and low overall mortality rate. This study also highlights the lack of standardised preventative pharmacogenomic measures and in vitro/in vivo diagnostic strategies to ascertain drug causality.
UNASSIGNED: ANZCTR ACTRN12619000241134. Registered 19 February 2019.