Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
J Eur Acad Dermatol Venereol. 2013 Jan;27(1):e17-23. doi: 10.1111/j.1468-3083.2011.04391.x. Epub 2011 Dec 17.
Pemphigus is a potentially fatal autoimmune epidermal bullous disorder. Various treatment modalities have been described to treat pemphigus. In cases where the disease fails to respond to conventional therapy, rituximab has been shown to be effective.
To study the efficacy of rituximab in the treatment of resistant or severe pemphigus in Indian patients.
Patients with pemphigus were treated with intravenous rituximab 1000 mg in adults or 375 mg/m(2) body surface area in children by two doses, 15 days apart in this open labelled pilot study. Anti-desmoglein1 (anti-Dsg1) antibodies and anti-desmoglein3 (anti-Dsg3) antibodies were measured at the start of therapy and at the end of the follow-up period. The outcome was studied in terms of control of disease activity (CD), complete remission (CR), partial remission (PR) and time to disease control (TDC) as defined by the consensus statement from the International Pemphigus Committee.
A total of 9 (90%) of 10 patients responded to the treatment. Three (30%) had CR of disease and were off all treatment. Four (40%) patients had CR and were on low dose oral prednisolone. Two (20%) patients had PR and were on low dose prednisolone. One patient died of sepsis. The mean TDC was 8 weeks. Response to treatment showed good correlation with index values of anti-Dsg1 antibody. Infusion-related angioedema and sepsis were seen as complications due to rituximab administration.
Rituximab is effective in treating resistant and severe pemphigus in Indian patients. Acute complications can occur during rituximab infusion and require close monitoring.
天疱疮是一种潜在致命的自身免疫性表皮大疱性疾病。已经描述了各种治疗方法来治疗天疱疮。在疾病对常规治疗无反应的情况下，利妥昔单抗已被证明是有效的。
研究利妥昔单抗治疗印度患者耐药或严重天疱疮的疗效。
在这项开放标签的初步研究中，将静脉注射利妥昔单抗成人 1000mg 或儿童 375mg/m(2)体表面积，分为两剂，间隔 15 天，用于治疗天疱疮患者。在治疗开始时和随访结束时测量抗桥粒芯糖蛋白 1（抗-Dsg1）抗体和抗桥粒芯糖蛋白 3（抗-Dsg3）抗体。根据国际天疱疮委员会的共识声明，以疾病活动控制（CD）、完全缓解（CR）、部分缓解（PR）和疾病控制时间（TDC）来研究结局。
共有 10 名患者中的 9 名（90%）对治疗有反应。3 名（30%）患者疾病完全缓解（CR）并停止所有治疗。4 名（40%）患者 CR 并接受低剂量口服泼尼松龙治疗。2 名（20%）患者 PR 并接受低剂量泼尼松龙治疗。1 名患者死于脓毒症。平均 TDC 为 8 周。治疗反应与抗-Dsg1 抗体的指数值有良好的相关性。输注相关血管性水肿和脓毒症是利妥昔单抗给药的并发症。
利妥昔单抗治疗印度患者的耐药和严重天疱疮有效。在利妥昔单抗输注期间可能发生急性并发症，需要密切监测。
