Correspondence

Rituximab for Pemphigus Vulgaris

N Engl J Med 2007; 356:521-522February 1, 2007

Article

To the Editor:

Ahmed et al. (Oct. 26 issue)1 report a resolution of pemphigus in 9 of 11 patients with refractory disease treated with a combination of 10 infusions of rituximab and 6 of intravenous immune globulin. On the basis of our experience with more than 20 patients with severe pemphigus who were treated with only one cycle of four infusions of rituximab,2 we confirm that rituximab has a dramatic effect: more than 90% of our patients had a complete remission, which was maintained after a 24-month follow-up period in two thirds of the patients. We did not treat the patients with intravenous immune globulin, and we observed only two cases of late infections. A single cycle of rituximab should be compared with the combination regimen proposed by Ahmed et al. in a clinical trial.

Pascal Joly, M.D., Ph.D.
Rouen University Hospital, 76000 Rouen, France
pascal.joly@chu-rouen.fr

Michel D'Incan, M.D., Ph.D.
Clermont-Ferrand University Hospital, 63000 Clermont-Ferrand, France

Philippe Musette, M.D., Ph.D.
Rouen University Hospital, 76000 Rouen, France

2 References

1. 1

   Ahmed AR, Spigelman Z, Cavacini LA, Posner MR. Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med 2006;355:1772-1779
   Full Text | Web of Science | Medline

2. 2

   Musette P, Mouquet H, Jacquot S, et al. Study of the B- and T-lymphocyte responses in pemphigus patients treated by anti-CD20 immunotherapy (rituximab). J Invest Dermatol 2006;126:Suppl 3:S23-S23
   CrossRef | Web of Science

Author/Editor Response

On the basis of the comments by Joly et al. about their patients with pemphigus, the patients treated with our published regimen appear to have fared better, although without the clinical details of their study in its entirety, it is difficult to make valid comparisons.1 Important questions with respect to their study include the definition of “severe pemphigus,” the duration of disease, immunosuppressive therapies used before rituximab therapy, and the ability to discontinue immunosuppressive therapies after rituximab therapy. The observations by Joly et al. appear to be similar to those in a report on 17 patients with pemphigus vulgaris who were treated with rituximab.2 We were able to discontinue all immunosuppressive agents in our patients before the cessation of rituximab therapy, and the patients have not needed them since. The role of intravenous immune globulin therapy is more than as prophylaxis against acute infections. The immunomodulatory effects of the drug may be synergistic with and complementary to rituximab in producing lasting periods of remission by preventing the reappearance of pathogenic B-cell clones and blocking the stimulation of T-cell help.

Pemphigus is an excellent model for investigating autoimmunity and providing insights into the pathogenesis of more complex autoimmune diseases. The availability of this effective therapy should lead to randomized trials that will contribute to this process.

Marshall R. Posner, M.D.
Dana–Farber Cancer Institute, Boston, MA 02115

Zachary Spigelman, M.D.
A. Razzaque Ahmed, M.D.
New England Baptist Hospital, Boston, MA 02120

2 References

1. 1

   Musette P, Mouquet H, Jacquot S, et al. Study of the B- and T-lymphocyte responses in pemphigus patients treated by anti-CD20 immunotherapy (rituximab). J Invest Dermatol 2006;126:Suppl 3:S23-S23
   CrossRef | Web of Science

2. 2

   El Tal AK, Posner MR, Spigelman Z, Ahmed AR. Rituximab: a monoclonal antibody to CD20 used in the treatment of pemphigus vulgaris. J Am Acad Dermatol 2006;55:449-459
   CrossRef | Web of Science | Medline

Citing Articles (7)

Citing Articles

1. 1

   Donna A. Culton, Luis A. Diaz. (2012) Treatment of subepidermal immunobullous diseases. Clinics in Dermatology 30:1, 95-102
   CrossRef

2. 2

   S. Lourari, C. Herve, V. Doffoel-Hantz, N. Meyer, C. Bulai-Livideanu, R. Viraben, A. Maza, D. Adoue, C. Bedane, C. Paul. (2011) Bullous and mucous membrane pemphigoid show a mixed response to rituximab: experience in seven patients. Journal of the European Academy of Dermatology and Venereology 25:10, 1238-1240
   CrossRef

3. 3

   Pascal Joly, Noémie Litrowski. (2011) Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clinics in Dermatology 29:4, 432-436
   CrossRef

4. 4

   Hugo Mouquet, Philippe Musette, Marie-Lyse Gougeon, Serge Jacquot, Brigitte Lemercier, Annick Lim, Danièle Gilbert, Ingrid Dutot, Jean C Roujeau, Michel D'Incan, Christophe Bedane, François Tron, Pascal Joly. (2008) B-Cell Depletion Immunotherapy in Pemphigus: Effects on Cellular and Humoral Immune Responses. Journal of Investigative Dermatology 128:12, 2859-2869
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5. 5

   Dagmar Simon, Susanne Hösli, Ganna Kostylina, Nikhil Yawalkar, Hans-Uwe Simon. (2008) Anti-CD20 (rituximab) treatment improves atopic eczema. Journal of Allergy and Clinical Immunology 121:1, 122-128
   CrossRef

6. 6

   H. Lapeyre, P. Joly. (2007) Rituximab : Mabthera®. Annales de Dermatologie et de Vénéréologie 134:12, 968-971
   CrossRef

7. 7

   Yoe Kie Onno Teng, Jacob M van Laar. (2007) Anticyclic citrullinated peptide antibodies: the footprint of autoreactive plasma cells in synovium?. Future Rheumatology 2:6, 577-586
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