Efficacy of additional i.v. immunoglobulin to steroid therapy in Stevens-Johnson syndrome and toxic epidermal necrolysis
Efficacy of additional i.v. immunoglobulin to steroid therapy in Stevens-Johnson syndrome and toxic epidermal necrolysis
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DOI：
10.1111/1346-8138.12925
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发表时间：
2015-08-01
影响因子：
3.1
通讯作者：
Shiohara, Tetsuo
中科院分区：
文献类型：
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作者：
Aihara, Michiko;Kano, Yoko;Shiohara, Tetsuo
Stevens-Johnson综合征（SJS）和中毒性表皮坏死松解症（TEN）是罕见且危及生命的皮肤药物不良反应。虽然SJS/TEN尚无既定治疗方法，但全身性皮质类固醇、血浆置换和静脉注射免疫球蛋白（IVIG）已被用作治疗。IVIG的有效性仍然存在争议，因为IVIG的总剂量在不同的研究中差异很大。本研究的目的是在SJS或TEN患者中进行一项开放标签、多中心、单组研究，评价IVIG连续给药5天的疗效。IVIG（400 mg/kg/d）连续5d给药，作为SJS或TEN成人患者全身类固醇治疗的补充。评价IVIG第7天的疗效。评估临床结局的参数为包括眼部和口腔病变、皮肤病变和一般状况在内的全身检查。在IVIG之前和之后对这些参数进行评分和记录。我们招募了5名SJS患者和3名TEN患者，这些患者在IVIG给药前对全身性类固醇反应不充分。所有患者均存活，IVIG第7天的有效率为87.5%（7/8例）。在IVIG治疗有效的患者中，观察到皮肤病变和糜烂迅速改善。未观察到使用IVIG的严重副作用。IVIG（400 mg/kg/d）连续5d治疗SJS或TEN有效。IVIG与类固醇联合给药应被视为难治性SJS/TEN患者的治疗方式。需要进一步的研究来确定IVIG的治疗效果。
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and life-threatening cutaneous adverse drug reactions. While there is no established therapy for SJS/TEN, systemic corticosteroids, plasma exchange and i.v. immunoglobulin (IVIG) have been used as treatment. The efficacy of IVIG is still controversial because total doses of IVIG used vary greatly from one study to another. The aim of this study was to evaluate the efficacy of IVIG, administrated for 5days consecutively, in an open-label, multicenter, single-arm study in patients with SJS or TEN. IVIG (400mg/kg per day) administrated for 5days consecutively was performed as an additional therapy to systemic steroids in adult patients with SJS or TEN. Efficacy on day 7 of IVIG was evaluated. Parameters to assess clinical outcome were enanthema including ophthalmic and oral lesions, cutaneous lesions and general condition. These parameters were scored and recorded before and after IVIG. We enrolled five patients with SJS and three patients with TEN who did not respond sufficiently to systemic steroids before IVIG administration. All of the patients survived and the efficacy on day 7 of the IVIG was 87.5% (7/8 patients). Prompt amelioration was observed in skin lesions and enanthema in the patients in whom IVIG therapy was effective. Serious side-effects from the use of IVIG were not observed. IVIG (400mg/kg per day) administrated for 5days consecutively seems to be effective in patients with SJS or TEN. IVIG administrated together with steroids should be considered as a treatment modality for patients with refractory SJS/TEN. Further studies are needed to define the therapeutic efficacy of IVIG.