Recommendations for the use of rituximab (anti‐CD20 antibody) in the treatment of autoimmune bullous skin diseases^§

Abstract

SummaryAutoimmune bullous skin disorders are induced by autoantibodies against distinct adhesion complexes of the epidermal and dermal-epidermal junction. Since most of these disorders are characterized by a severe, potentially lethal course, they require long-term immunosuppressive treatment to reduce the de novo synthesis of pathogenic autoantibodies by B lymphocytes. Rituximab, a chimeric monoclonal antibody against CD20 on B lymphocytes, has shown promise in several case reports or cohort studies in the treatment of paraneoplastic pemphigus, refractory cases of pemphigus vulgaris and foliaceus and in other autoimmune bullous disorders.Treatment with rituximab leads to depletion of pathogenic B-cells which may last up to 12 months resulting in a reduction of plasma cells secreting pathogenic autoantibodies. Rituximab is usually administered in an adjuvant setting at a dose of 375 mg/m 2 i. v. in weekly intervals for four consecutive weeks in addition to the standard immunosuppressive treatment. The present consensus statement of German-speaking dermatologists, rheumatologists and oncologists summarizes and evaluates the current evidence for the use and mode of application of rituximab in autoimmune bullous skin disorders.

Pemphigus. S2 Guideline for diagnosis and treatment – guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV)

“…[22][23][24][25] • A course of intravenous rituximab 2 9 1000 mg (2 weeks apart or 4 9 375 m2/1 week apart). The need for immunosuppressive adjuvants in rituximab therapy remains unclear…”

A brief review of pemphigus vulgaris

“…Rituximab can curb the disease, especially in refractory cases. Several case studies have shown rituximab to be efficacious (Hertl et al 2008;Arin et al 2005). Cyclophosphamide is also used in refractory cases, but it has an unsafe side effect profile (Saha et al 2009).…”

Pemfigusta Konvansiyonel Olmayan Tedavi Yaklaşımları

“…Gebe ve emziren kadınlarda, yetersiz klinik deneyimden dolayı 18 yaş altı hastalarda, aktif hepatit b ve c hastalığı ile HIV pozitif olanlarda ve kontrol edilemeyen infeksiyon ile şiddetli kalp yetmezliği olan hastalarda kullanımı kontrendikedir (25).…”

“…B hücrelerindeki azalmayı takip etmek için son infüzyondan sonra her üç ayda bir kan sayımı yapılması önerilmektedir. Nötrofil sayısı 1.5x 10 9 /l ve/veya trombosit sayısı 75x10 9 /l olan hastalarda ritüksimab tedavisi dikkatli bir şekilde sürdürülmelidir (25). …”