Teschke Rolf
Department of Internal Medicine II, Division of Gastroenterology and Hepatology, Klinikum Hanau, D-63450 Hanau, Academic Teaching Hospital of the Medical Faculty, Goethe University Frankfurt, Frankfurt/Main, Germany.
J Clin Transl Hepatol. 2025 Apr 28;13(4):339-357. doi: 10.14218/JCTH.2024.00402. Epub 2025 Jan 17.
Liver injury in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) is a multifaceted disorder, lacking cohort homogeneity due to a variety of potential causes, including drugs, arsenic and other heavy metals, glyphosate, infections, and ultraviolet radiation. The goals of this review were (1) to analyze the role of diagnostic algorithms in assessing causality for potential culprits involved in the development of liver injury associated with immune-mediated SJS and TEN, which represent immune-based variant disorders within a continuous spectrum. Milder forms are classified as SJS or SJS/TEN overlap, while TEN is known as the most serious form; and (2) to interpret the findings that allow for the characterization of the different types of these disorders. The manuscript is based on an extensive literature search for single case reports, case cohorts, and review articles. Search terms included: Stevens-Johnson Syndrome, Toxic Epidermal Necrolysis, and specific diagnostic algorithms such as the Roussel Uclaf Causality Assessment Method (RUCAM) and the Algorithm of Drug Causality for Epidermal Necrolysis (ALDEN). For the purpose of basic feature description, the uniform term SJS/TEN is used in the current analysis. SJS/TEN presents with five different cohort types: SJS/TEN type (1), which refers to a cohort of SJS/TEN caused by drugs, as assessed by both ALDEN and RUCAM; type (2), representing SJS/TEN due to drugs and assessed by ALDEN only, but not by RUCAM; type (3), which includes a cohort of SJS/TEN caused by drugs, assessed by non-ALDEN and non-RUCAM tools; type (4), which focuses on a cohort of SJS/TEN caused by non-drug culprits, assessed by various tools; and type (5), which considers a cohort of SJS/TEN caused by unknown culprits. Using this new SJS/TEN typology will help better characterize individual features, personalize treatment, and clarify pathogenetic specifics for each of the five disease types. This new SJS/TEN typology provides clarity by replacing issues of inhomogeneity with cohort homogeneity.
史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）中的肝损伤是一种多方面的病症，由于多种潜在病因，包括药物、砷及其他重金属、草甘膦、感染和紫外线辐射，缺乏队列同质性。本综述的目的是：（1）分析诊断算法在评估与免疫介导的SJS和TEN相关的肝损伤发展中潜在罪魁祸首的因果关系方面的作用，SJS和TEN代表连续谱系内基于免疫的变异病症。较轻的形式归类为SJS或SJS/TEN重叠，而TEN是最严重的形式；（2）解读有助于对这些病症不同类型进行特征描述的研究结果。该手稿基于对单病例报告、病例队列和综述文章的广泛文献检索。检索词包括：史蒂文斯-约翰逊综合征、中毒性表皮坏死松解症以及特定的诊断算法，如鲁塞尔·优克福因果关系评估方法（RUCAM）和表皮坏死松解症药物因果关系算法（ALDEN）。为进行基本特征描述，本分析中使用统一术语SJS/TEN。SJS/TEN呈现出五种不同的队列类型：SJS/TEN类型（1），指经ALDEN和RUCAM评估由药物引起的SJS/TEN队列；类型（2），代表由药物引起且仅经ALDEN评估而非RUCAM评估的SJS/TEN；类型（3），包括经非ALDEN和非RUCAM工具评估由药物引起的SJS/TEN队列；类型（4），关注由非药物罪魁祸首引起且经各种工具评估的SJS/TEN队列；类型（5），考虑由不明罪魁祸首引起的SJS/TEN队列。使用这种新的SJS/TEN分类法将有助于更好地描述个体特征、个性化治疗，并阐明五种疾病类型中每种类型的发病机制细节。这种新的SJS/TEN分类法通过用队列同质性取代异质性问题，提供了清晰度。
