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Effect of a Single-Cycle Alternative Dosing Regimen for Rituximab for Recalcitrant Pemphigus:  A Case Series of 9 Patients

Setsuko Matsukura, MD, PhD; Sandra R. Knowles, BScPhm; Scott Walsh, MD, PhD; Neil H. Shear, MD
Arch Dermatol. 2012;148(6):734-739. doi:10.1001/archdermatol.2011.3320.
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Background  There is increasing evidence that a single cycle of rituximab (375 mg per square meter of body surface area once weekly for 4 weeks) is efficacious in patients with severe pemphigus. The approved protocol in rheumatoid arthritis is 1 g on days 1 and 15. We report herein on the efficacy and safety of this latter protocol for rituximab in 9 patients with pemphigus.

Observations  Nine patients with recalcitrant pemphigus were treated with prednisone, immunosuppressive agents, and/or intravenous immunoglobulin. Rituximab, 1 g, was infused on days 1 and 15. Each patient was observed for a minimum of 6 months. Reepithelialization of at least 50% of the affected areas occurred in all patients within 16 weeks. Three of 6 patients (50%) discontinued intravenous immunoglobulin therapy. A significant decrease in the pemphigus severity score and the mean dosage of prednisone was observed at 3 and 6 months. Relapses were observed in 4 patients between 5 and 13 months after rituximab treatment; these patients completed a second cycle of rituximab. There were no serious adverse effects observed during the follow-up period.

Conclusions  A single cycle of rituximab, 1 g on days 1 and 15, is an effective treatment for pemphigus. Further studies are needed to determine the efficacy and safety of repeated treatment courses in patients who experience recurrences.

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Figures

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Figure 1. Pemphigus severity score before and after rituximab treatment. A significant decrease (P < .05) was observed in the mean score (red curve) of 9 patients at 3 and 6 months compared with the mean score at baseline.

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Figure 2. Effect of rituximab treatment on the prednisone dosage in 8 patients. A, Most had a dramatic decrease in the prednisone dosage except 1 patient who discontinued its use at 3 months but started again at 6 months because of disease recurrence. B, A significant (P < .05 [*]) decrease (↓) in the mean prednisone dosage at 3 and 6 months was observed compared with the mean dosage at baseline. The percentage of change in the prednisone dosage was measured from baseline.

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Figure 3. Change in the dosage of each immunosuppressive agent after rituximab treatment in 8 of our 9 patients. A, Mycophenolate mofetil hydrochloride. B, Azathioprine sodium and cyclophosphamide in patients 5 and 9, respectively. C, Methotrexate sodium in patient 7. Our usual protocol is to taper the corticosteroid dosage first and then decrease or withdraw intravenous immunoglobulin therapy followed by immunosuppressive agent tapering. Most patients stayed on the same dosage of their immunosuppressive agent during the study period.

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