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环孢素与支持性治疗在史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症中的有效性、安全性和耐受性:一项基于记录的研究。

Effectiveness, safety and tolerability of cyclosporine versus supportive treatment in Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A record-based study.

作者信息

Mohanty Swosti, Das Anupam, Ghosh Anupama, Sil Amrita, Gharami Ramesh Chandra, Bandyopadhyay Debabrata, Das Nilay Kanti

机构信息

Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India.

Department of Pharmacology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India.

出版信息

Indian J Dermatol Venereol Leprol. 2017 May-Jun;83(3):312-316. doi: 10.4103/ijdvl.IJDVL_201_16.

DOI:10.4103/ijdvl.IJDVL_201_16
PMID:28366923
Abstract

BACKGROUND

Toxic epidermal necrolysis and Stevens-Johnson syndrome comprise life-threatening, drug-induced mucocutaneous disease spectrum. Interest in cyclosporine, a calcineurin inhibitor that can block the function of T-cells, has increased with the discovery of the importance of granulysin in apoptosis in toxic epidermal necrolysis. In our hospital, cyclosporine is given to Stevens-Johnson syndrome/toxic epidermal necrolysis patients as an adjunctive therapy.

AIMS

This study is an observational, record-based study comparing the effectiveness and safety of patients receiving cyclosporine versus only supportive therapy.

METHODOLOGY

Medical records as bed-head tickets and laboratory investigation reports of Stevens-Johnson syndrome/toxic epidermal necrolysis patients admitted in the hospital over a period of 1 year were collected. Data regarding clinico-demographic profile, suspected drug causing Stevens-Johnson's syndrome/toxic epidermal necrolysis, SCORTEN, body surface area involved, treatment received and outcome were obtained.

RESULTS

Twenty-eight patients were analyzed. Nineteen belonged to the cyclosporine group (supportive treatment + cyclosporine), nine to supportive treatment only group. Among the suspected drugs, antiepileptics formed the major group (28.6%). Five patients in the supportive only group and one in the cyclosporine group died. Time for stabilization and reepithelialization and duration of recovery were significantly lower in the cyclosporine group (P < 0.001, P= 0.007, P= 0.01, respectively). The standardized mortality ratio was 0.32 in cyclosporine group which is nearly 3.3 times lower than the only supportive treatment.

LIMITATIONS

As it was a record-based study, certain confounding factors (serum blood urea nitrogen) could not be adjusted.

CONCLUSION

Cyclosporine (5 mg/kg/day) for 10 days from onset of Stevens-Johnson syndrome/toxic epidermal necrolysis may decrease the risk of dying, may provide faster healing of lesions and might lead to early discharge from hospital.

摘要

背景

中毒性表皮坏死松解症和史蒂文斯-约翰逊综合征属于危及生命的药物性皮肤黏膜疾病谱。随着颗粒溶素在中毒性表皮坏死松解症细胞凋亡中的重要性被发现,人们对环孢素(一种可阻断T细胞功能的钙调神经磷酸酶抑制剂)的兴趣增加。在我们医院,环孢素被用作史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症患者的辅助治疗药物。

目的

本研究是一项基于记录的观察性研究,比较接受环孢素治疗的患者与仅接受支持性治疗的患者的有效性和安全性。

方法

收集了1年内入院的史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症患者的床头病历和实验室检查报告等医疗记录。获取了有关临床人口统计学特征、疑似导致史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症的药物、SCORTEN、受累体表面积、接受的治疗和结局的数据。

结果

对28例患者进行了分析。19例属于环孢素组(支持性治疗+环孢素),9例属于仅支持性治疗组。在疑似药物中,抗癫痫药占主要部分(28.6%)。仅支持性治疗组有5例患者死亡,环孢素组有1例患者死亡。环孢素组的病情稳定时间、上皮再生时间和恢复持续时间显著更短(分别为P < 0.001、P = 0.007、P = 0.01)。环孢素组的标准化死亡率为0.32,比仅支持性治疗组低近3.3倍。

局限性

由于这是一项基于记录的研究,某些混杂因素(血清血尿素氮)无法进行调整。

结论

史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症发病后10天内使用环孢素(5mg/kg/天)可能会降低死亡风险,可能使病变愈合更快,并可能导致患者早日出院。

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