Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin
Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin
复制标题
DOI：
10.1056/nejmoa062930
复制
发表时间：
2006-10-26
影响因子：
158.5
通讯作者：
Posner, Marshall R.
中科院分区：
文献类型：
--
作者：
Ahmed, A. Razzaque;Spigelman, Zachary;Posner, Marshall R.
背景：寻常型天疱疮是一种潜在的致命性自身免疫性皮肤粘膜水疱性疾病。常规治疗包括高剂量的皮质类固醇，免疫抑制剂，静脉注射免疫球蛋白。方法：我们研究了难治性寻常天疱疮患者涉及30%或更多的体表面积，三个或更多的粘膜网站，或两者都有不足的反应，常规治疗和静脉注射免疫球蛋白。我们治疗患者两个周期的利妥昔单抗（375毫克每平方米的体表面积）每周一次，为期3周，静脉注射免疫球蛋白（2克每公斤体重）在第四周。在诱导治疗后，每月输注利妥昔单抗和静脉注射免疫球蛋白，连续4个月。对角质形成细胞和外周血B细胞的数量的血清抗体滴度monitored.Results：11例患者中，9例迅速解决的病变和临床缓解持续22至37个月（平均31.1）。所有患者在结束利妥昔单抗治疗前，可以停止所有免疫抑制治疗，包括泼尼松。两名患者仅在复发期间接受利妥昔单抗治疗，并获得持续缓解。IgG 4抗角质形成细胞抗体滴度与疾病活动相关。外周血B细胞在开始利妥昔单抗治疗后不久变得不可检测，但随后恢复到正常值。与利妥昔单抗相关的副作用没有观察到，也没有infections.Conclusions：利妥昔单抗和静脉注射免疫球蛋白的组合是有效的难治性寻常天疱疮患者。
BACKGROUND:Pemphigus vulgaris is a potentially fatal autoimmune mucocutaneous blistering disease. Conventional therapy consists of high-dose corticosteroids, immunosuppressive agents, and intravenous immune globulin.METHODS:We studied patients with refractory pemphigus vulgaris involving 30% or more of their body-surface area, three or more mucosal sites, or both who had inadequate responses to conventional therapy and intravenous immune globulin. We treated the patients with two cycles of rituximab (375 mg per square meter of body-surface area) once weekly for 3 weeks and intravenous immune globulin (2 g per kilogram of body weight) in the fourth week. This induction therapy was followed by a monthly infusion of rituximab and intravenous immune globulin for 4 consecutive months. Titers of serum antibodies against keratinocytes and numbers of peripheral-blood B cells were monitored.RESULTS:Of 11 patients, 9 had rapid resolution of lesions and a clinical remission lasting 22 to 37 months (mean, 31.1). All immunosuppressive therapy, including prednisone, could be discontinued before ending rituximab treatment in all patients. Two patients were treated with rituximab only during recurrences and had sustained remissions. Titers of IgG4 antikeratinocyte antibodies correlated with disease activity. Peripheral-blood B cells became undetectable shortly after initiating rituximab therapy but subsequently returned to normal values. Side effects that have been associated with rituximab were not observed, nor were infections.CONCLUSIONS:The combination of rituximab and intravenous immune globulin is effective in patients with refractory pemphigus vulgaris.