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Adjuvant Rituximab Therapy of Pemphigus:  A Single-Center Experience With 31 Patients

Luisa Lunardon, MD; Kathleen J. Tsai; Kathleen J. Propert, ScD; Nicole Fett, MD; John R. Stanley, MD; Victoria P. Werth, MD; Donald E. Tsai, MD, PhD; Aimee S. Payne, MD, PhD
Arch Dermatol. 2012;148(9):1031-1036. doi:10.1001/archdermatol.2012.1522.
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Background  We conducted a retrospective study of patients with pemphigus vulgaris (n = 24) and foliaceus (n = 7) treated with adjuvant rituximab to determine efficacy and adverse events. The end point for efficacy was complete remission of disease taking no or minimal therapy.

Observations  Eighteen patients (58%) achieved the study end point. Of these, 13 patients achieved complete remission off systemic therapy. Patients achieving the study end point had a median disease duration before rituximab therapy of 19 months vs 86 months in those not achieving the end point (P = .01). For the 18 patients achieving the end point, the median (SD) duration of remission was 19 (2) months. Eight of these 18 patients (44%) relapsed from 6 to 17 months after treatment. Serious adverse events attributed to rituximab treatment (osteomyelitis or phlegmon) occurred in 2 patients (6%). In paired serum samples from 10 patients before and after rituximab treatment, the percent change in serum desmoglein index value (median, −80%) was unrelated to the percent change in pneumococcal antibodies (median, +8%) (Spearman rank correlation coefficient r = −0.2).

Conclusions  Patients treated with rituximab earlier in the course of disease may have better outcomes. A discussion of rituximab's mechanism of action supports the rationale for early therapy. Prospective clinical studies are necessary to substantiate this observation.

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Grahic Jump Location

Figure 1. Complete remission of disease on no or minimal therapy is associated with use of rituximab earlier in the course of disease. Median (interquartile range) disease duration before rituximab therapy was 19 (14-41) months for patients achieving the study end point compared with 86 (55-144) months in those not achieving the study end point (P = .01 by Wilcoxon rank sum test). The horizontal line in the middle of each box indicates the median, while the top and bottom borders of the box mark the 75th and 25th percentiles, respectively. The whiskers above and below the box mark the 90th and 10th percentiles. The point beyond the whiskers is an outlier beyond the 90th percentile.

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Grahic Jump Location

Figure 2. Kaplan-Meier plot of time to relapse after rituximab infusion in patients achieving complete remission (n = 18). Circles indicate duration of follow-up for patients remaining in complete remission (n = 10). The median (SD) duration of remission was 19 (2) months. Dotted lines indicate the 95% CI.

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Grahic Jump Location

Figure 3. Changes from before to after rituximab therapy in serum levels of desmoglein and pneumococcal capsular polysaccharide antibodies were unrelated (n = 10; Spearman rank correlation coefficient r = −0.2). A median change of −80% in desmoglein index value vs +8% in pneumococcal antibody concentration was observed.

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