Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin.
BACKGROUND
Pemphigus vulgaris is a potentially fatal autoimmune mucocutaneous blistering disease. Conventional therapy consists of high-dose corticosteroids, immunosuppressive agents, and intravenous immune globulin.


METHODS
We studied patients with refractory pemphigus vulgaris involving 30% or more of their body-surface area, three or more mucosal sites, or both who had inadequate responses to conventional therapy and intravenous immune globulin. We treated the patients with two cycles of rituximab (375 mg per square meter of body-surface area) once weekly for 3 weeks and intravenous immune globulin (2 g per kilogram of body weight) in the fourth week. This induction therapy was followed by a monthly infusion of rituximab and intravenous immune globulin for 4 consecutive months. Titers of serum antibodies against keratinocytes and numbers of peripheral-blood B cells were monitored.


RESULTS
Of 11 patients, 9 had rapid resolution of lesions and a clinical remission lasting 22 to 37 months (mean, 31.1). All immunosuppressive therapy, including prednisone, could be discontinued before ending rituximab treatment in all patients. Two patients were treated with rituximab only during recurrences and had sustained remissions. Titers of IgG4 antikeratinocyte antibodies correlated with disease activity. Peripheral-blood B cells became undetectable shortly after initiating rituximab therapy but subsequently returned to normal values. Side effects that have been associated with rituximab were not observed, nor were infections.


CONCLUSIONS
The combination of rituximab and intravenous immune globulin is effective in patients with refractory pemphigus vulgaris.
Ahmed
A Razzaque
AR
Center for Blistering Diseases, New England Baptist Hospital, Boston, USA.
Spigelman
Zachary
Z
Cavacini
Lisa A
LA
Posner
Marshall R
MR
D000293:Adolescent; D000328:Adult; D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies / Q000097:blood; D001402:B-Lymphocytes; D051182:Desmogleins / Q000276:immunology; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D015603:Keratinocytes / Q000276:immunology; D018655:Lymphocyte Count; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy* / Q000276:immunology; D012008:Recurrence; D012074:Remission Induction; D000069283:Rituximab
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D051182:Desmogleins; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D000069283:Rituximab