Rituximab therapy for refractory autoimmune bullous diseases: A multicenter, open-label, single-arm, phase 1/2 study on 10 Japanese patients

Yuichi Kurihara, Jun Yamagami, Takeru Funakoshi, Maki Ishii, Julia Miyamoto, Yumi Fujio, Risa Kakuta, Akiko Tanikawa, Yumi Aoyama, Keiji Iwatsuki, Norito Ishii, Takashi Hashimoto, Wataru Nishie, Hiroshi Shimizu, Keisuke Koyama, Masayuki Amagai

Research output: Contribution to journalArticle

Abstract

This was a multicenter study of rituximab, a chimeric monoclonal immunoglobulin G antibody directed against CD20, for the treatment of refractory autoimmune bullous diseases (pemphigus and pemphigoid). Ten patients (three with pemphigus vulgaris, six with pemphigus foliaceus and one with bullous pemphigoid) were treated with a single cycle of rituximab (four weekly infusions at a dose of 375 mg/m2 of body surface area). The primary end-points were the number of serious adverse events and rate of complete remission at 40 weeks. Five patients (50%) achieved complete remission with minimal therapy (defined as no active lesions with lower doses of systemic corticosteroids compared to that with prednisolone 10 mg/day). Improvements in clinical scores (Pemphigus Disease Area Index) and decreases in autoantibody titers in the sera were observed in the four pemphigus patients who failed to achieve complete remission. This suggests that rituximab was effective in nine of 10 cases. Two serious adverse events (Pneumocystis carinii pneumonia and septic shock due to infectious arthritis) were observed and adequately treated with hospitalization. CD19-positive B lymphocytes in the peripheral blood decreased on day 29 following rituximab treatment, and remained at low levels throughout the observation period (280 days). Our results confirmed the efficacy of rituximab therapy for refractory autoimmune bullous diseases in Japan.

Original languageEnglish
JournalJournal of Dermatology
DOIs
Publication statusAccepted/In press - 2018 Jan 1

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Pemphigus
Autoimmune Diseases
Bullous Pemphigoid
Therapeutics
Infectious Arthritis
Pneumocystis Pneumonia
Body Surface Area
Septic Shock
Prednisolone
Autoantibodies
Multicenter Studies
Japan
Adrenal Cortex Hormones
Hospitalization
B-Lymphocytes
Immunoglobulin G
Observation
Rituximab
Antibodies
Serum

Keywords

  • CD20 antigens
  • immunotherapy
  • pemphigoid
  • pemphigus
  • rituximab

ASJC Scopus subject areas

  • Dermatology

Cite this

Rituximab therapy for refractory autoimmune bullous diseases : A multicenter, open-label, single-arm, phase 1/2 study on 10 Japanese patients. / Kurihara, Yuichi; Yamagami, Jun; Funakoshi, Takeru; Ishii, Maki; Miyamoto, Julia; Fujio, Yumi; Kakuta, Risa; Tanikawa, Akiko; Aoyama, Yumi; Iwatsuki, Keiji; Ishii, Norito; Hashimoto, Takashi; Nishie, Wataru; Shimizu, Hiroshi; Koyama, Keisuke; Amagai, Masayuki.

In: Journal of Dermatology, 01.01.2018.

Research output: Contribution to journalArticle

Kurihara, Yuichi ; Yamagami, Jun ; Funakoshi, Takeru ; Ishii, Maki ; Miyamoto, Julia ; Fujio, Yumi ; Kakuta, Risa ; Tanikawa, Akiko ; Aoyama, Yumi ; Iwatsuki, Keiji ; Ishii, Norito ; Hashimoto, Takashi ; Nishie, Wataru ; Shimizu, Hiroshi ; Koyama, Keisuke ; Amagai, Masayuki. / Rituximab therapy for refractory autoimmune bullous diseases : A multicenter, open-label, single-arm, phase 1/2 study on 10 Japanese patients. In: Journal of Dermatology. 2018.
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AU - Funakoshi, Takeru

AU - Ishii, Maki

AU - Miyamoto, Julia

AU - Fujio, Yumi

AU - Kakuta, Risa

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AU - Iwatsuki, Keiji

AU - Ishii, Norito

AU - Hashimoto, Takashi

AU - Nishie, Wataru

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