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史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症:亚太地区视角

Stevens-Johnson syndrome / toxic epidermal necrolysis: an Asia-Pacific perspective.

作者信息

Thong Bernard Yu-Hor

机构信息

Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore 308433, Singapore.

出版信息

Asia Pac Allergy. 2013 Oct;3(4):215-23. doi: 10.5415/apallergy.2013.3.4.215. Epub 2013 Oct 31.

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions (SCAR) to drugs which are associated with significant morbidity and mortality. High risk drugs in Asia are similar to those reported worldwide. Human leukocyte antigen (HLA)-related risk alleles for carbamazepine and allopurinol SCAR are unique to Asians. Although prognostic scoring systems like the SCORTEN have been used for more than a decade, pitfalls and caveats need to be recognized, in particular in patients with multiple medical co-morbidities and systemic features in SJS/TEN. In centres without a tertiary Burns Centre, SJS/TEN patients can still be managed successfully in general and dermatology wards with well-executed supportive/nursing care. Controversy remains regarding the effectiveness of immunomodulation in reducing SJS/TEN morbidity, mortality and hastening re-epithelialization. Despite paucity of robust evidence, intravenous immunoglobulins and ciclosporin remain the most commonly used modalities worldwide. Acute and long-term ocular effects are an important source of morbidity for which emerging ophthalmic therapies appear promising. Quality of life issues have now become an important outcome in patients with SJS/TEN as they often impact survivors' future attitudes towards pharmacotherapy. Even though pharmacogenetic testing for high-risk drugs appears to be the panacea for preventing carbamazepine- and allopurinol-induced SJS/TEN in ethnic Asians, many issues remain before health regulators in our region can conclusively determine whether testing should be made mandatory or highly recommended as standard of care.

摘要

史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)是药物引起的严重皮肤不良反应(SCAR),与显著的发病率和死亡率相关。亚洲的高风险药物与全球报告的类似。卡马西平和别嘌醇SCAR的人类白细胞抗原(HLA)相关风险等位基因在亚洲人中是独特的。尽管像SCORTEN这样的预后评分系统已经使用了十多年,但仍需认识到其缺陷和注意事项,特别是在患有多种合并症以及SJS/TEN有全身症状的患者中。在没有三级烧伤中心的医院,SJS/TEN患者在普通病房和皮肤科病房通过精心实施的支持性/护理措施仍可得到成功治疗。关于免疫调节在降低SJS/TEN发病率、死亡率以及加速上皮再形成方面的有效性仍存在争议。尽管缺乏有力证据,但静脉注射免疫球蛋白和环孢素仍然是全球最常用的治疗方式。急性和长期眼部影响是发病率的一个重要来源,新兴的眼科治疗方法似乎很有前景。生活质量问题现在已成为SJS/TEN患者的一个重要结局,因为它们常常影响幸存者未来对药物治疗的态度。尽管对高风险药物进行药物遗传学检测似乎是预防亚洲人种中卡马西平和别嘌醇引起的SJS/TEN的万灵药,但在我们地区的卫生监管机构能够最终确定是否应强制进行检测或强烈推荐将其作为标准治疗方法之前,仍有许多问题需要解决。

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