Severe Delayed Cutaneous and Systemic Reactions to Drugs: A Global Perspective on the Science and Art of Current Practice.
Severe Delayed Cutaneous and Systemic Reactions to Drugs: A Global Perspective on the Science and Art of Current Practice.
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DOI：
10.1016/j.jaip.2017.01.025
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发表时间：
2017-05
期刊：
影响因子：
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通讯作者：
Phillips EJ
中科院分区：
文献类型：
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作者：
Peter JG;Lehloenya R;Dlamini S;Risma K;White KD;Konvinse KC;Phillips EJ
大多数免疫介导的药物不良反应（im - adr）涉及皮肤，并且许多具有额外的全身特征。严重皮肤药物不良反应（SCAR）是一种罕见的、可能危及生命的、具有挑战性的im - adr亚组，具有多种临床表型、机制和致病药物。t细胞介导的免疫病理是这些严重延迟反应的核心，但效应细胞和细胞因子因临床表型而异。hla基因与特异性药物- scar im - adr（如Stevens-Johnson综合征/中毒性表皮坏死松解症（SJS/TEN））的强烈关联已被阐明；尽管特定HLA等位基因的携带对于许多im - adr的发展是必要的但不是充分的机制仍在定义中。由于大量的短期和长期发病率/死亡率，以及可能需要用相关药物治疗持续的合并症，SCAR的管理变得复杂。有经验的单位的多学科专家团队应该照顾病人。在SCAR的情况下，患者的预后以及预防、诊断、治疗和管理方法往往不是一般的，而是具体的，受人群hla遗传学、相关药物的药理学和遗传风险因素、需要持续治疗的潜在合并症的严重程度以及成本考虑的驱动。在本文中，我们对SCAR诊断和治疗的基础和临床科学进行了综述。
The majority of immune-mediated adverse drug reactions (IM-ADRs) involve the skin, and many have additional systemic features. Severe cutaneous adverse drug reactions (SCAR) are an uncommon, potentially life-threatening and challenging sub-group of IM-ADRs with diverse clinical phenotypes, mechanisms and offending drugs. T-cell mediated immunopathology is central to these severe delayed reactions, but effector cells and cytokines differ by clinical phenotype. Strong HLA-gene associations have been elucidated for specific drug-SCAR IM-ADRs such as Stevens-Johnson Syndrome/toxic epidermal necrolysis (SJS/TEN); although the mechanisms by which carriage of a specific HLA allele is necessary but not sufficient for the development of many IM-ADRs is still being defined. SCAR management is complicated by substantial short and long-term morbidity/ mortality and the potential need to treat ongoing co-morbid disease with related medications. Multidisciplinary specialist teams at experienced units should care for patients. In the setting of SCAR, patient outcomes as well as preventive,diagnostic, treatment and management approaches are often not generalizable, but rather context specific, driven by population HLA-genetics, the pharmacology and genetic risk factors of the implicated drug, severity of underlying co-morbid disease necessitating ongoing treatments, and cost considerations. In this review, we update the basic and clinical science of SCAR diagnosis and management.