The Australasian Registry for Severe Cutaneous Adverse Reactions (AUS-SCAR) – Providing a roadmap for closing the diagnostic, patient, and healthcare gaps for a group of rare drug eruptions

IF 3.9 2区医学 Q2 ALLERGY

World Allergy Organization Journal Pub Date : 2024-08-01 DOI:10.1016/j.waojou.2024.100936

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引用次数: 0

Abstract

Background

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.

Methods

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.

Results

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%).

Conclusion

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.

Trial registration

ANZCTR ACTRN12619000241134. Registered 19 February 2019.

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澳大拉西亚严重皮肤不良反应登记处（AUS-SCAR）--为缩小一组罕见药疹在诊断、患者和医疗保健方面的差距提供路线图

背景严重皮肤不良反应（SCAR）是一组假定由 T 细胞介导的延迟性过敏反应，与严重的发病率和死亡率有关。尽管它们对全球医疗保健造成了共同的负担和影响，但其临床表型、基因组易感性、药物因果关系和治疗结果可能各不相同。我们介绍了首个澳大拉西亚 SCAR 登记处（AUS-SCAR）的建立和成果，该登记处通过合作网络推进 SCAR 的预防、诊断和治疗策略。该登记处收集经外部验证的表型数据、药物因果关系、治疗方法和患者的长期疗效。结果我们报告了澳大利亚-SCAR 数据库首批登记的 100 名患者的情况。DRESS（50%）是最主要的表型，其次是SJS/TEN（39%）和AGEP（10%），中位年龄为52岁（IQR为37.5-66岁），男女比例为1:1。所有涉及药物的中位潜伏期差异很大，但 DRESS（中位数 15 天，IQR 5,25）和 SJS/TEN（中位数 21 天，IQR 7,27）的潜伏期相似，而 AGEP（中位数 2.5 天，IQR 1,8）的潜伏期最低。与非抗生素药物（45.5%）相比，抗生素（54.5%）更常被列为主要牵连药物。结论在南半球首个前瞻性全国 SCAR 表型和生物资料库中，我们发现了与其他登记报告的显著差异；包括以 DRESS 为主导的表型、不同的抗生素因果关系和较低的总死亡率。这项研究还凸显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的因果关系。试验注册ANZCTR ACTRN12619000241134。注册日期：2019年2月19日。

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来源期刊

World Allergy Organization Journal Immunology and Microbiology-Immunology

CiteScore

9.10

自引率

5.90%

发文量

审稿时长

9 weeks

期刊介绍： The official pubication of the World Allergy Organization, the World Allergy Organization Journal (WAOjournal) publishes original mechanistic, translational, and clinical research on the topics of allergy, asthma, anaphylaxis, and clincial immunology, as well as reviews, guidelines, and position papers that contribute to the improvement of patient care. WAOjournal publishes research on the growth of allergy prevalence within the scope of single countries, country comparisons, and practical global issues and regulations, or threats to the allergy specialty. The Journal invites the submissions of all authors interested in publishing on current global problems in allergy, asthma, anaphylaxis, and immunology. Of particular interest are the immunological consequences of climate change and the subsequent systematic transformations in food habits and their consequences for the allergy/immunology discipline.