Saeed Hajirah N, Chodosh James
a Massachusetts Eye and Ear Infirmary, Department of Ophthalmology, Harvard Medical School , Boston , Massachusetts , USA.
Semin Ophthalmol. 2016;31(1-2):85-90. doi: 10.3109/08820538.2015.1115255.
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are a spectrum of T-cell mediated immune disorders. While the contributory mechanisms leading to the apoptosis of epidermal cells in SJS/TEN remain unproven, the keratinocyte apoptosis seen in SJS/TEN is thought to occur through the T-cell mediated Fas-Fas ligand (FasL), perforin/granzyme B, and other immune mediators. Most recently, emphasis has been placed on the granulysin pathway as being the primary mediator of apoptosis and widespread epidermal necrosis in SJS/TEN. This article aims to review the proposed mechanisms by which these pathways work and the immunomodulatory therapies that have been developed in an attempt to target them.
史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)是一系列由T细胞介导的免疫紊乱疾病。虽然导致SJS/TEN中表皮细胞凋亡的具体机制尚未得到证实,但SJS/TEN中所见的角质形成细胞凋亡被认为是通过T细胞介导的Fas-Fas配体(FasL)、穿孔素/颗粒酶B及其他免疫介质发生的。最近,人们将重点放在颗粒溶素途径上,认为它是SJS/TEN中细胞凋亡和广泛表皮坏死的主要介质。本文旨在综述这些途径发挥作用的假定机制以及为靶向这些机制而研发的免疫调节疗法。