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Multicenter Study
. 2025 Apr 1;161(4):399-405.
doi: 10.1001/jamadermatol.2024.6130.

Optimizing Pemphigus Management With Rituximab and Short-Term Relapse Predictors

Vivien Hébert  1 , Sami Hamwi  1 , Emmanuelle Tancrède-Bohin  2 , Gaelle Quéreux  3 , Anne Pham-Ledard  4 , Frédéric Caux  5 , Billal Tedbirt  1 , Alexis Lefebvre  1 , Nadège Cordel  6 , Marina Alexandre  5 , Manuelle Viguier  7 , Géraldine Jeudy  8 , Michel D'Incan  9 , Sébastien Debarbieux  10 , Alexis Brue  11 , Sophie Duvert-Lehembre  12 , Marion Fenot  13 , Vannina Seta  14 , Saskia Ingen-Housz-Oro  15   16   17 , Clémence Lepelletier  2 , Pascal Joly  1
Affiliations
Multicenter Study

Optimizing Pemphigus Management With Rituximab and Short-Term Relapse Predictors

Vivien Hébert et al. JAMA Dermatol. .

Erratum in

  • Error in Abstract.
    [No authors listed] [No authors listed] JAMA Dermatol. 2025 Apr 1;161(4):450. doi: 10.1001/jamadermatol.2025.0875. JAMA Dermatol. 2025. PMID: 40238109 Free PMC article. No abstract available.
  • Group Omitted From Article Byline.
    [No authors listed] [No authors listed] JAMA Dermatol. 2025 Jul 1;161(7):776. doi: 10.1001/jamadermatol.2025.1555. JAMA Dermatol. 2025. PMID: 40434766 Free PMC article. No abstract available.

Abstract

Importance: Rituximab is approved for the treatment of moderate to severe pemphigus. However, 20% of patients in the RITUX 3 trial relapsed within the first year of treatment.

Objective: To assess the outcome of an additional rituximab infusion at month 6 in patients with pemphigus who were in complete remission (CR) after rituximab regimen but had 1 or more predictors of relapse at month 3.

Design, settings, and participants: This multicenter cohort study was conducted in France from September 2018 to June 2023 to assess patients with newly diagnosed pemphigus who were in CR after treatment with the RITUX 3 regimen but had predictors of relapse at month 3. Relapse factors were a Pemphigus Disease Area Index (PDAI) score of 45 or higher, desmoglein 1 (DSG1) antibodies greater than 20 IU/mL, and/or DSG3 antibodies greater than 130 IU/mL.

Exposure: Patients in CR at month 6 with at least 1 predictor of relapse were treated with an additional rituximab infusion at month 6.

Main outcomes and measures: Primary end point was the rate of CR without corticosteroid therapy for 2 months at month 12. Secondary end points were the rate of relapse, number of patients needing to be re-treated (NNT) with rituximab to avoid a relapse, and safety.

Results: The study population comprised 87 patients (44 females [50.6%] and 43 [49.4%] males), with a mean (SD [range]) age of 55.3 (15.2 [24-92]) years at pemphigus diagnosis. Of these, 64 patients (73.6%) had pemphigus vulgaris and 23 (26.4%) had pemphigus foliaceus. At month 6, CR had been achieved by 77 patients (88.5%), and 10 (11.5%) had persistent disease activity. Of the 77 patients in CR, 30 (39.0%) had at least 1 predictor of relapse and received an additional infusion of rituximab; 47 patients (61.0%) without a predictor did not. Two patients without a predictor and no patients with a predictor experienced relapse-an overall relapse rate of 2.6% and an NNT of 3.6 (95% CI, 1.6-46.5). The 10 patients (11.5%) with persistent disease activity at month 6 were re-treated with rituximab, 2000 mg. At month 12, the rate of CR without corticosteroid therapy for a minimum of 2 months was 72 of 77 (93.5%) among patients who had achieved CR at month 6, and 72 of 87 (82.7%) for the whole study population. Eight serious adverse effects were reported among 5 patients; there were no deaths.

Conclusion and relevance: This multicenter cohort study indicates that using predictors such as baseline PDAI score, anti-DSG1 antibodies, and/or anti-DSG3 antibodies to initiate preemptive treatment with additional rituximab may reduce the rate of short-term relapse.

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Conflict of interest statement

Conflict of Interest Disclosures: Dr Hébert reported consulting fees from Lilly, Janssen, AbbVie, Pfizer, and Almirall outside the submitted work. Dr Caux reported research support and/or consulting fees from Argenx, Principia Biopharma, Regeneron, and Sanofi during the conduct of the study. No other disclosures were reported.

References

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