Efficacy and safety of rituximab in pemphigus: experience of the German Registry of Autoimmune Diseases

  1. Michael Kasperkiewicz1,†,
  2. Rüdiger Eming2,†,
  3. Melika Behzad2,
  4. Nicolas Hunzelmann3,
  5. Michael Meurer4,
  6. Hendrik Schulze-Koops5,
  7. Peter von Wussow6,
  8. Michael Hertl2,
  9. Detlef Zillikens1,
  10. Klaus Freivogel7,
  11. Thomas Dörner8,
  12. Enno Schmidt1,9

Article first published online: 11 MAY 2012

DOI: 10.1111/j.1610-0387.2012.07931.x

Issue

JDDG: Journal der Deutschen Dermatologischen Gesellschaft

JDDG: Journal der Deutschen Dermatologischen Gesellschaft

Volume 10, Issue 10, pages 727–731, October 2012

Additional Information

How to Cite

Kasperkiewicz, M., Eming, R., Behzad, M., Hunzelmann, N., Meurer, M., Schulze-Koops, H., von Wussow, P., Hertl, M., Zillikens, D., Freivogel, K., Dörner, T. and Schmidt, E. (2012), Efficacy and safety of rituximab in pemphigus: experience of the German Registry of Autoimmune Diseases. JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 10: 727–731. doi: 10.1111/j.1610-0387.2012.07931.x

Author Information

  1. 1

     Department of Dermatology, University of Lübeck, Germany

  2. 2

     Department of Dermatology and Allergology, Philipp University Marburg, Germany

  3. 3

     Department of Dermatology, University of Cologne, Germany

  4. 4

     Department of Dermatology, Dresden University of Technology, Germany

  5. 5

     Division of Rheumatology, University of Munich, Germany

  6. 6

     Division of Hematology and Oncology, Hospital Siloah, Hannover, Germany

  7. 7

     United BioSource Corporation, Lörrach, Germany

  8. 8

     Charité Center 12 and 14, Charité University Hospital & Deutsches Rheuma-Forschungszentrum Berlin, Germany

  9. 9

     Comprehensive Center for Inflammation Medicine, University of Lübeck, Germany

  1. †

    equal contribution

*Enno Schmidt, MD, PhD, Department of Dermatology, University of Lübeck, Ratzeburger Allee 160, D-23538 Lübeck, Germany. Tel.: +49-451-500-2538 Fax: +49-451-500-8129 E-mail: enno.schmidt@uk-sh.de

  1. †

    equal contribution

Publication History

  1. Issue published online: 26 SEP 2012
  2. Article first published online: 11 MAY 2012
  3. Submitted: 4.1.2012 | Accepted: 20.2.2012

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Keywords:

  • autoantibody;
  • bullous disease;
  • pemphigus vulgaris;
  • registry

Summary

Background: Rituximab has been reported to be effective in various small case series of patients with severe and/or refractory pemphigus. However, no systematic evaluation is available to corroborate this observation. The aim of this study was to systematically determine efficacy and safety of rituximab in treatment-resistant pemphigus.

Patients and Methods: Multicenter retrospective, observational study of 36 patients with severe pemphigus vulgaris (n = 33) and pemphigus foliaceus (n = 3) treated with rituximab before August 31st, 2008 and enrolled in a national observational registery between December 2008 and June 2009.

Results: Within a mean period of observation of 11 (1–37) months, 21 (58 %) pemphigus patients showed complete, 13 (36 %) partial, and 2 (6 %) no response to rituximab treatment. This correlates with a mean improvement of the visual analog scale for well-being of 34 (20–60) at baseline to 75 (40–95) at the last control visit. In 4 (11 %) patients, severe adverse events were recorded including 1 (3 %) serious infection.

Conclusions: Data collected in this systematic registry indicate that rituximab is an effective and relatively safe adjuvant treatment option for refractory pemphigus. To further extend our knowledge on efficacy and safety of this drug, controlled prospective trials are required.

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