Get access

Efficacy and safety of rituximab treatment in Indian pemphigus patients

Authors

  • A.J. Kanwar,

    Corresponding author
    1. Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
    Search for more papers by this author
  • D. Tsuruta,

    1. Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Fukuoka, Japan
    Search for more papers by this author
  • K. Vinay,

    1. Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
    Search for more papers by this author
  • H. Koga,

    1. Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Fukuoka, Japan
    Search for more papers by this author
  • N. Ishii,

    1. Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Fukuoka, Japan
    Search for more papers by this author
  • T. Dainichi,

    1. Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Fukuoka, Japan
    Search for more papers by this author
  • T. Hashimoto

    Corresponding author
    1. Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Fukuoka, Japan
    Search for more papers by this author

  • Few case series have shown efficacy of rituximab in resistant cases of pemphigus. There are individual case reports of use of rituximab in childhood pemphigus.

  • We have shown the efficacy of rituximab in the Indian pemphigus patients, for the first time. Clinical response showed good correlation with fall in anti-Dsg1 antibody indices, whereas there was only a partial reduction in anti-Dsg3 indices. Childhood pemphigus also responded to rituximab therapy.

  • Funding source
    This study was supported by Grants-in-Aid for Scientific Research and Strategic Research Basis Formation Supporting Project from the Ministry of Education, Culture, Sports, Science and Technology of Japan, and by Health and Labour Sciences Research Grants and grants for Research on Measures for Intractable Diseases from the Ministry of Health, Labour and Welfare of Japan. The study was also supported by grants from the Uehara Memorial Foundation, Nakatomi Foundation, Kaibara Morikazu Medical Science Promotion Foundation, Japan Lydia O’Leary Memorial Foundation, Cosmetology Research Foundation, Japanese Dermatological Association (Shiseido Award), Fukuoka Foundation for Sound Health, and Galderma K.K. (Galderma Award).

  • Conflict of interest
    None.

*A.J. Kanwar, T. Hashimoto. E-mails:ajkanwar1948@gmail.com; hashimot@med.kurume-u.ac.jp

Abstract

Background  Pemphigus is a potentially fatal autoimmune epidermal bullous disorder. Various treatment modalities have been described to treat pemphigus. In cases where the disease fails to respond to conventional therapy, rituximab has been shown to be effective.

Objective  To study the efficacy of rituximab in the treatment of resistant or severe pemphigus in Indian patients.

Methods  Patients with pemphigus were treated with intravenous rituximab 1000 mg in adults or 375 mg/m2 body surface area in children by two doses, 15 days apart in this open labelled pilot study. Anti-desmoglein1 (anti-Dsg1) antibodies and anti-desmoglein3 (anti-Dsg3) antibodies were measured at the start of therapy and at the end of the follow-up period. The outcome was studied in terms of control of disease activity (CD), complete remission (CR), partial remission (PR) and time to disease control (TDC) as defined by the consensus statement from the International Pemphigus Committee.

Results  A total of 9 (90%) of 10 patients responded to the treatment. Three (30%) had CR of disease and were off all treatment. Four (40%) patients had CR and were on low dose oral prednisolone. Two (20%) patients had PR and were on low dose prednisolone. One patient died of sepsis. The mean TDC was 8 weeks. Response to treatment showed good correlation with index values of anti-Dsg1 antibody. Infusion-related angioedema and sepsis were seen as complications due to rituximab administration.

Conclusion  Rituximab is effective in treating resistant and severe pemphigus in Indian patients. Acute complications can occur during rituximab infusion and require close monitoring.

Ancillary