Center for Blistering Diseases, 697 Cambridge Street 302, Boston, MA 02135 USA.
Dermatol Ther (Heidelb). 2012 Dec;2(1):17. doi: 10.1007/s13555-012-0017-3. Epub 2012 Nov 15.
Rituximab is increasingly used in patients with pemphigus vulgaris (PV) who are nonresponders to conventional therapy.
A PubMed search was conducted using the words pemphigus vulgaris and rituximab therapy from papers published between 2000 and 2012. Two protocols were used. In the lymphoma protocol, patients received four weekly infusions of rituximab (dose 375 mg/m(2)). The rheumatoid arthritis (RA) protocol consisted of two infusions of 1,000 mg each 15 days apart. The variables recorded from each study included clinical remission off or on therapy, relapse rate, incidence of serious adverse events, concomitant therapies, duration of follow-up, and when available, levels of B cells and autoantibodies.
Forty-two studies were found, which reported 272 patients; 180 were treated by the lymphoma protocol and 92 by the RA protocol. Both protocols were effective in treating recalcitrant PV. The lymphoma protocol had a lower response rate, relapse rate and serious infections, but higher mortality, and there were nonresponders. The RA protocol produced a higher response rate, relapse rate, number of infections, but lower mortality rate, and lacked nonresponders. The cumulative follow-up for patients treated with the lymphoma protocol was 15.44 months (range 1-41) and 21.04 months (range 8.35-29) for the RA protocol. A major concern in both protocols was the high infection rates, some of which were fatal. A different protocol using a combination of rituximab with intravenous immunoglobulin in a defined manner with a definitive endpoint, used in a limited cohort of patients, showed promising results.
Neither protocol produced a sustained clinical remission and both required continued systemic therapy. Before initiation of treatment, physicians should have a specific goal and endpoint and be aware of its potential side effects and lack of information on its long-term effects. Patients should be carefully monitored during and after therapy.
利妥昔单抗在寻常型天疱疮（PV）患者中越来越多地被用于对传统治疗无反应的患者。
使用从 2000 年至 2012 年发表的文献中使用“寻常型天疱疮”和“利妥昔单抗治疗”这两个词在 PubMed 上进行了搜索。使用了两种方案。在淋巴瘤方案中，患者接受每周四次的利妥昔单抗输注（剂量 375mg/m(2)）。类风湿关节炎（RA）方案由两次输注组成，每次 1000mg，间隔 15 天。从每项研究中记录的变量包括治疗期间和治疗后的临床缓解、复发率、严重不良事件的发生率、伴随治疗、随访时间以及在可能的情况下，B 细胞和自身抗体的水平。
发现 42 项研究，报告了 272 例患者；180 例患者接受淋巴瘤方案治疗，92 例患者接受 RA 方案治疗。两种方案均能有效治疗难治性 PV。淋巴瘤方案的反应率、复发率和严重感染率较低，但死亡率较高，且有治疗无应答者。RA 方案的反应率、复发率、感染次数较高，但死亡率较低，且无治疗无应答者。接受淋巴瘤方案治疗的患者的累积随访时间为 15.44 个月（范围 1-41），接受 RA 方案治疗的患者为 21.04 个月（范围 8.35-29）。两个方案都存在一个主要问题，即高感染率，其中一些感染是致命的。一项使用利妥昔单抗联合静脉注射免疫球蛋白以特定方式、具有明确终点的不同方案，在有限的患者队列中显示出了良好的效果。
两种方案均未产生持续的临床缓解，且均需要持续的系统治疗。在开始治疗之前，医生应该有一个明确的目标和终点，并了解其潜在的副作用以及缺乏长期效果的信息。在治疗期间和治疗后，应对患者进行仔细监测。
