Rituximab in the Treatment of Pemphigus Vulgaris

Abstract

IntroductionRituximab is increasingly used in patients with pemphigus vulgaris (PV) who are nonresponders to conventional therapy.MethodsA 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/m2). 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.ResultsForty-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.ConclusionNeither 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.

The Management of Pemphigus Vulgaris in a Burn Intensive Care Unit

“…At a mean 18-month follow-up, complete remission was achieved in 66.66% of those patients on the lymphoma protocol and in 75% of those on the rheumatoid arthritis protocol. 32 An additional 12.78% and 23.91% demonstrated partial remission on the lymphoma and the rheumatoid arthritis protocol, respectively. 32 More study is needed to determine the optimum dosing of rituximab, with the 2012 review finding no distinct advantage to using either the lymphoma or the rheumatoid arthritis protocol.…”

“…32 An additional 12.78% and 23.91% demonstrated partial remission on the lymphoma and the rheumatoid arthritis protocol, respectively. 32 More study is needed to determine the optimum dosing of rituximab, with the 2012 review finding no distinct advantage to using either the lymphoma or the rheumatoid arthritis protocol. The review also noted that the incidence of serious infection was 3.9% in the lymphoma protocol and 15.21% in the rheumatoid arthritis protocol with a respective mortality rate of 2.22% and 1.09%.…”

Efficacy and safety of rituximab in the treatment of refractory pemfigus vulgaris

“…Bu grupta tedaviye yanıtsız hasta olmadığı belirtilmiştir. Çalışma sonunda her iki protokolde de en büyük endişenin ciddi enfeksiyon riskindeki artış olduğu belirtilmiştir 25 . Çalışmamızda anti-Dsg antikor seviyeleri düşmesine rağmen hastaların hiçbirinde enfeksiyon gelişimi gözlenmemiştir.…”

Pemfigusta Konvansiyonel Olmayan Tedavi Yaklaşımları

“…Lenfoma protokolünde dört haftalık ritüksimab infüzyonu (375mg/m 2 /hafta) söz konusu iken romatoid artrit protokolünde ise 15 gün arayla 1000mg'lık iki infüzyon yapılır (24). Zakka ve arkadaşlarının yaptıkları ve ritüksimab tedavisi verilen pemfigus vulgarisli hastalarla ilgili literatür taramasını konu alan derlemede 272 hastanın yer aldığı toplam 42 çalışmadan bahsedilmiştir.…”

“…Bildirilen olguların 180' inde lenfoma protokolü kullanılmış iken 92' sinde romatoid artrit protokolü uygulanmıştır. Lenfoma protokolü uygulanan hastalarda daha düşük klinik düzelme oranı tespit edilmiş buna karşın enfeksiyon riski daha düşük bulunmuştur (24).…”