关键词: 中毒性表皮松解症;stevens-Johnson综合症;皮质类固醇激素;静脉注射丙种球蛋白;联合治疗;SCORTEN

Abstract:

Objective   To compare the efficacy of combination therapy of intravenous immunoglobulin(IVIG) and corticosteroids versus corticosteroids alone in the treatment of toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS). Methods   A retrospective study was conducted. Totally, 65 consecutive patients diagnosed as either TEN or SJS from January 1993 to October 2007 were included in this study. For 45 patients collected from 1993 to 2000, including 35 cases of TEN and 10 cases of SJS, methylprednisolone of 1 - 1.5 mg per kilogram bodyweight per day or equivalent hydrocortisone or dexamethasone was given; for the remaining 20 patients collected from 2001 to 2007, including 12 cases of TEN and 8 cases of SJS, additional IVIG (0.4 g·kg-1·d-1 for 5 days)was given. The efficacy was evaluated based on SCORTEN, a severity-of-illness-score system for TEN/SJS prognosis. Results   Among the 45 patients treated with corticosteroids alone, 8.63 patients were expected to die based on SCORTEN system, while 10 deaths were observed. Standardized mortality ratio (SMR) analysis revealed that the patients treated with corticosteroids alone were 16% more likely to die than those treated with routine therapy (SMR = 1.16; 95% confidence interval, 0.56 - 2.13). In the remaining 20 patients who received combination therapy, 3 deaths occurred, while 3.51 deaths were expected based on the SCORTEN system. SMR analysis showed that the combination therapy had a trend to reduce the mortality rate of TEN/SJS (SMR = 0.85; 95% confidence interval, 0.18 - 2.50). No significant difference was noted in the mortality rate of TEN/SJS between the combination therapy and corticosteroid monotherapy (16.7% vs 22.8% in TEN, 12.5% vs 20% in SJS, respectively, both P > 0.05). In patients with TEN, the combination therapy significantly reduced the time to arrest disease progression （4.30 ± 2.36 days vs 7.15 ± 3.35 days, t = 2.46, P < 0.05） and total hospitalized time （23.40 ± 5.10 days vs 34.30 ± 16.00 days, t = 3.14, P < 0.05）, but had no significant effect on the time to taper corticosteroid dose（12.30 ± 3.10 days vs 12.20 ± 5.13 days, t = -0.045, P > 0.05） compared with the corticosteroid monotherapy did; so was the case for SJS. Conclusions   Compared with solo administration of corticosteroids, the combination therapy with corticosteroid and IVIG seems to reduce the mortality rate of TEN/SJS. Also, the combination therapy could arrest the progression of TEN/SJS earlier accompanied by a decrease in hospitalization time. However, the time period of corticosteroid tapering is not shortened by the combination therapy.