Background

Drug reaction with eosinophilia and systemic symptoms (DRESS) is an intractable drug hypersensitivity disease with high mortality. The current standard treatment requires high-dose and long-term systemic corticosteroids, which may lead to adverse effects and intolerability of patients.

Objective

To evaluate the efficacy and safety of cyclosporine in patients with corticosteroid-dependent DRESS or intolerance to systemic corticosteroids.

Methods

A retrospective review of 8 patients with corticosteroid-dependent DRESS who were treated with cyclosporine as an alternative treatment owing to suboptimal response to regular doses of systemic corticosteroids for at least 3 weeks, flare-ups when tapering corticosteroids, or experiencing intolerable adverse effects of corticosteroids.

Results

In all 8 patients (4 women and 4 men; age range, 15-75 years), either intractable skin eruptions, persistent eosinophilia, or elevated liver function was noted after at least 3 weeks of treatment with systemic corticosteroids. The patients had marked cutaneous improvement and normalization of liver function and eosinophil count after adding cyclosporine, and the systemic corticosteroid treatment was smoothly tapered down. The mean dosage of cyclosporine was 1.68 ± 0.73 mg/kg/d, and the mean duration of cyclosporine treatment was 76.13 ± 35.64 days. Their serum eosinophil counts, serum alanine aminotransferase levels, and serum thymus and activation-regulated chemokine levels were all elevated at baseline and then significantly decreased during the recovery stage after cyclosporine therapy (P < .05). No adverse events were reported after adding cyclosporine.

Conclusion

Cyclosporine is an effective and safe therapeutic alternative as a steroid-sparing agent for corticosteroid-dependent DRESS. Further prospective randomized controlled studies are required to confirm these preliminary results.

中文翻译：

---

用环孢素成功治疗皮质类固醇依赖性药物反应伴嗜酸性粒细胞增多和全身症状

背景

嗜酸性粒细胞增多症和全身症状药物反应（DRESS）是一种难治性药物超敏反应性疾病，死亡率很高。目前的标准治疗需要大剂量长期全身使用皮质类固醇，可能导致患者出现不良反应和不耐受。

客观的

评估环孢素对皮质类固醇依赖性 DRESS 或对全身性皮质类固醇不耐受的患者的疗效和安全性。

方法

对 8 例皮质类固醇依赖型 DRESS 患者的回顾性研究，这些患者因对常规剂量全身性皮质类固醇至少 3 周反应欠佳、逐渐减量皮质类固醇或经历皮质类固醇不可耐受的副作用而接受环孢素作为替代治疗.

结果

在所有 8 名患者（4 名女性和 4 名男性；年龄范围，15-75 岁）中，在接受全身性皮质类固醇治疗至少 3 周后，均出现顽固性皮疹、持续性嗜酸性粒细胞增多或肝功能升高。加用环孢素后，患者皮肤明显改善，肝功能和嗜酸性粒细胞计数正常化，全身糖皮质激素治疗逐渐减量。环孢素的平均剂量为 1.68 ± 0.73 mg/kg/d，环孢素治疗的平均持续时间为 76.13 ± 35.64 天。他们的血清嗜酸性粒细胞计数、血清丙氨酸氨基转移酶水平、血清胸腺和激活调节趋化因子水平均在基线时升高，然后在环孢素治疗后的恢复期显着降低（P< .05)。加用环孢素后未报告任何不良事件。

结论

环孢菌素是一种有效且安全的治疗替代品，可作为皮质类固醇依赖型 DRESS 的类固醇节约剂。需要进一步的前瞻性随机对照研究来证实这些初步结果。