Pemphigus is a rare autoimmune disease of the skin, characterized by autoantibodies targeting adhesion proteins of the epidermis, in particular desmoglein 3 and desmoglein 1, that cause the loss of cell-cell adhesion and the formation of intraepidermal blisters. Given that these autoantibodies are both necessary and sufficient for pemphigus to occur, the goal of pemphigus therapy is the elimination of autoreactive Bcells responsible for autoantibody production. Rituximab, an anti-CD20 monoclonal antibody, was the first targeted B-cell therapy approved for use in pemphigus, and is now considered the frontline therapy for new onset disease. One limitation of this treatment is that it targets both autoreactive and non - autoreactive B-cells, which accounts for the increased risk of serious infections in treated patients. In addition, most rituximab-treated patients experience disease relapse, highlighting the need of new therapeutic options. This review provides a concise overview of rituximab use in pemphigus and discusses new B-cell and antibody-directed therapies undergoing investigation in clinical studies.

B-cell targeted therapies in pemphigus / Maglie, Roberto; Antiga, Emiliano; Payne, Aimee S. - In: ITALIAN JOURNAL OF DERMATOLOGY AND VENEREOLOGY. - ISSN 2784-8450. - STAMPA. - 156(2021), pp. 161-173. [10.23736/S0392-0488.20.06694-8]

B-cell targeted therapies in pemphigus

Maglie, Roberto;Antiga, Emiliano;
2021

Abstract

Pemphigus is a rare autoimmune disease of the skin, characterized by autoantibodies targeting adhesion proteins of the epidermis, in particular desmoglein 3 and desmoglein 1, that cause the loss of cell-cell adhesion and the formation of intraepidermal blisters. Given that these autoantibodies are both necessary and sufficient for pemphigus to occur, the goal of pemphigus therapy is the elimination of autoreactive Bcells responsible for autoantibody production. Rituximab, an anti-CD20 monoclonal antibody, was the first targeted B-cell therapy approved for use in pemphigus, and is now considered the frontline therapy for new onset disease. One limitation of this treatment is that it targets both autoreactive and non - autoreactive B-cells, which accounts for the increased risk of serious infections in treated patients. In addition, most rituximab-treated patients experience disease relapse, highlighting the need of new therapeutic options. This review provides a concise overview of rituximab use in pemphigus and discusses new B-cell and antibody-directed therapies undergoing investigation in clinical studies.
156
161
173
Goal 3: Good health and well-being
Maglie, Roberto; Antiga, Emiliano; Payne, Aimee S
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2158/1208405
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