title: Treatment of refractory pemphigus vulgaris with rituximab (anti-CD20 monoclonal antibody).
abstract: BACKGROUND
  Pemphigus vulgaris (PV) is a severe antibody-mediated autoimmune blistering disease. Because some patients with PV do not enter into remission, despite the use of high-dose corticosteroid therapy and immunosuppressive adjuvant treatments, new effective and safer agents are warranted to treat refractory PV. Rituximab, a monoclonal anti-CD20 antibody, induces depletion of B cells in vivo and has shown efficacy in patients with refractory antibody-mediated autoimmune disorders. We describe herein 3 patients treated with rituximab for severe PV.
  
  
  METHODS
  Three patients with refractory PV were treated with rituximab, resulting in a clinical response in all patients, which was complete in 2 patients. A decline in titers of circulating antiepidermis autoantibodies paralleled disease activity, while circulating B cells remained undetectable for 6 to 10 months. Two patients experienced bacterial infection in the weeks following the rituximab course. A clinical relapse occurred in 2 patients, at 6 and 10 months. A second course of rituximab controlled the disease in one of them.
  
  
  CONCLUSIONS
  These patients' response suggests that rituximab may be a valuable treatment for refractory PV and warrants further studies to evaluate the risk-benefit ratio in patients with PV showing resistance to classic therapy.
authors.affiliation: Institut de Recherche sur la Peau, Institut National de la Santé et de la Recherche Médicale Unité 532, and Service de Dermatologie 2, Laboratoire d'Immunologie, Hôpital Saint-Louis Assistance Publique-Hôpitaux de Paris, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Dupuy
authors.forename: Manuelle
authors.initials: M
authors.lastname: Viguier
authors.forename: Christophe
authors.initials: C
authors.lastname: Bédane
authors.forename: Florence
authors.initials: F
authors.lastname: Cordoliani
authors.forename: Sophie
authors.initials: S
authors.lastname: Blaise
authors.forename: Françoise
authors.initials: F
authors.lastname: Aucouturier
authors.forename: Jean-Marie
authors.initials: JM
authors.lastname: Bonnetblanc
authors.forename: Patrice
authors.initials: P
authors.lastname: Morel
authors.forename: Louis
authors.initials: L
authors.lastname: Dubertret
authors.forename: Hervé
authors.initials: H
authors.lastname: Bachelez
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies / Q000097:blood; D004817:Epidermis / Q000276:immunology; D005260:Female; D006801:Humans; D008297:Male; D010392:Pemphigus / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D000069283:Rituximab
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D000069283:Rituximab