REVIEWS  PEMPHIGUS 

Giornale Italiano di Dermatologia e Venereologia 2007 August;142(4):381-90

Copyright © 2007 EDIZIONI MINERVA MEDICA

language: English

The selective immunotherapy of pemphigus

Bystryn J. C., Czernik A.

The Ronald O. Perelman Department of Dermatology The New York University School of Medicine New York, NY, USA

Pemphigus is conventionally treated with systemic steroids often given in conjunction with immunosuppressive agents. There is a need for improved treatments as some patients fail to respond or develop severe complications to current therapies. Furthermore, even though pemphigus is caused by the production of a very limited set of abnormal autoantibodies, current therapy nonspecifically suppresses all immune responses, resulting in unnecessary toxicity. Two general approaches are currently being pursued to improve the treatment of pemphigus: 1) more potent, but still nonspecific, procedures to suppress the production of pemphigus antibodies, such as inactivating B cells with anti-CD20 antibodies or those that interfere with mediators involved in the disease process such as tumor necrosis factor; and 2) selective procedures which are intended to remove or prevent the production of only those antibodies which are pathogenic, such as immunoadsorption columns, pemphigus “vaccines” and intravenous immunoglobulin (IVIg). Evaluating the effectiveness of these new approaches is challenging, as patients are often treated concurrently with other therapies known to be effective in pemphigus, making it difficult to determine which therapy was responsible for any eventual improvement. All newer treatments are considerably more expensive than conventional therapy, and are often viewed as experimental, which limits their availability. The two approaches which have the strongest evidence of clinical effectiveness are anti-CD20 antibody and IVIg. Of the two, IVIg is particularly attractive as it results in the rapid and selective decrease in only pathogenic antibodies. The effectiveness of IVIg may be improved by coadministration of a cytotoxic agent to prevent the rebound in antibody levels that may otherwise occur.