Rituximab therapy for intractable pemphigus: A multicenter, open-label, single-arm, prospective study of 20 Japanese patients

Jun Yamagami, Yuichi Kurihara, Takeru Funakoshi, Yasuko Saito, Ryo Tanaka, Hayato Takahashi, Hideyuki Ujiie, Hiroaki Iwata, Yoji Hirai, Keiji Iwatsuki, Norito Ishii, Jun Sakurai, Takayuki Abe, Ryo Takemura, Naomi Mashino, Masahiro Abe, Masayuki Amagai

Research output: Contribution to journalArticlepeer-review

Abstract

This was a multicenter clinical trial of rituximab, a chimeric monoclonal IgG antibody directed against CD20, for the treatment of refractory pemphigus vulgaris and pemphigus foliaceus. In total, 20 patients were treated with two doses of rituximab (1000 mg; 2 weeks apart) on days 0 and 14. The primary end point was the proportion of patients who achieved complete or partial remission on day 168 following the first rituximab dose. Of the 20 enrolled patients, 11 (55%) and four (20%) achieved complete and partial remission, respectively; therefore, remission was achieved in a total of 15 patients (75.0% [95% confidence interval, 50.9%–91.3%]). It was demonstrated that the remission rate was greater than the prespecified threshold (5%). In addition, a significant improvement in clinical score (Pemphigus Disease Area Index) and decrease in serum anti-desmoglein antibody level were observed over time. Four serious adverse events (heart failure, pneumonia, radial fracture, and osteonecrosis) were recorded in two patients, of which only pneumonia was considered causally related with rituximab. The level of peripheral blood CD19-positive B lymphocytes was decreased on day 28 after rituximab treatment and remained low throughout the study period until day 168. Our results confirm the efficacy and safety of rituximab therapy for refractory pemphigus in Japanese patients.

Original languageEnglish
JournalJournal of Dermatology
DOIs
Publication statusAccepted/In press - 2022

Keywords

  • anti-desmoglein antibody
  • corticosteroid tapering
  • refractory pemphigus
  • rituximab

ASJC Scopus subject areas

  • Dermatology

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