Treatment of refractory pemphigus vulgaris with rituximab (anti-CD20 monoclonal antibody).
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.
Dupuy
Alain
A
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.
Viguier
Manuelle
M
Bédane
Christophe
C
Cordoliani
Florence
F
Blaise
Sophie
S
Aucouturier
Françoise
F
Bonnetblanc
Jean-Marie
JM
Morel
Patrice
P
Dubertret
Louis
L
Bachelez
Hervé
H
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
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D000069283:Rituximab