Treatment of refractory pemphigus with the anti-CD20 monoclonal antibody (rituximab).
BACKGROUND
Pemphigus is a severe blistering disorder caused by autoantibodies to desmogleins 1 and 3. Because some patients with pemphigus never enter into remission, new immunosuppressants are warranted. Rituximab is a chimeric monoclonal antibody binding to the CD20 antigen on B cells, which proved to be effective in recalcitrant pemphigus.


OBJECTIVE
To evaluate the efficacy and safety of rituximab in refractory pemphigus and to investigate its effects on the autoantibody profile.


METHODS
Six patients with recalcitrant pemphigus were treated. Rituximab was administered intravenously at a dosage of 375 mg/m2 body surface once weekly for 4 weeks.


RESULTS
Three pemphigus foliaceus patients and 1 with mucocutaneous pemphigus vulgaris (PV) showed complete response over a follow-up period of up to 18 months. In one oral PV, control of the disease was achieved using pulse therapy with cyclophosphamide following rituximab withdrawal. In one PV with vegetating features, good improvement was obtained after 6 rituximab infusions. All patients tolerated the treatment well. Anti-desmoglein autoantibodies significantly decreased only in pemphigus foliaceus.


CONCLUSIONS
This study highlights that rituximab is a valuable drug for refractory pemphigus, although the response of mucous membranes and cutaneous folds may be delayed.
Marzano
Angelo V
AV
Institute of Dermatological Sciences, University of Milan, IRCCS Ospedale Maggiore Policlinico, Mangiagalli and Regina Elena of Milan, Milan, Italy.
Fanoni
Daniele
D
Venegoni
Luigia
L
Berti
Emilio
E
Caputo
Ruggero
R
D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D018941:Antigens, CD19 / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D001402:B-Lymphocytes / Q000276:immunology; D051183:Desmoglein 1 / Q000097:blood; D051184:Desmoglein 3 / Q000097:blood; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy*; D000069283:Rituximab; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018941:Antigens, CD19; D001323:Autoantibodies; C071894:DSG3 protein, human; D051183:Desmoglein 1; D051184:Desmoglein 3; D007155:Immunologic Factors; D000069283:Rituximab