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Previous Volume 355:1772-1779 October 26, 2006 Number 17 Next

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ABSTRACT

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.

Source Information

From the Center for Blistering Diseases (A.R.A.), Parker Hill Oncology and Hematology (Z.S.), and the Department of Medicine (A.R.A., Z.S.), New England Baptist Hospital; the Department of Oral Medicine, Infection, and Immunity, Harvard School of Dental Medicine (A.R.A.); the Waltham Cancer Center and Harvard Medical School (Z.S.); the Human Monoclonal Antibody Laboratory, Beth Israel Deaconess Medical Center (L.A.C., M.R.P.); and Dana–Farber Cancer Institute and Harvard Medical School (M.R.P.) — all in Boston.

Address reprint requests to Dr. Ahmed at the Center for Blistering Diseases, New England Baptist Hospital, 70 Parker Hill Ave., Suite 208, Boston, MA 02120.

Full Text of this Article

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Rituximab for Pemphigus Vulgaris
Joly P., D'Incan M., Musette P., Posner M. R., Spigelman Z., Ahmed A. R.
Extract | Full Text | PDF  
N Engl J Med 2007; 356:521-522, Feb 1, 2007. Correspondence

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