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International Journal of Rheumatology
Volume 2011, Article ID 214013, 7 pages
http://dx.doi.org/10.1155/2011/214013
Review Article

B-Cell Depletion Therapy in Systemic Sclerosis: Experimental Rationale and Update on Clinical Evidence

Division of Rheumatology, Department of Internal Medicine, Patras University Hospital, University of Patras Medical School, Rion, 26504 Patras, Greece

Received 24 February 2011; Revised 4 June 2011; Accepted 7 June 2011

Academic Editor: Laura K. Hummers

Copyright © 2011 Dimitrios Daoussis et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Systemic sclerosis (SSc) is a systemic rheumatic disease with poor prognosis since therapeutic options are limited. Recent evidence from animal models suggests that B-cells may be actively involved in the fibrotic process. B-cells from tight skin mice, an animal model of scleroderma, display a “hyperresponsive” phenotype; treatment with rituximab (RTX) significantly attenuates skin fibrosis in this animal model. In humans, B-cell infiltration is a prominent finding in most lung biopsies obtained from patients with SSc-associated interstitial lung disease. Several open label studies have assessed the clinical efficacy of RTX in SSc. In most patients skin fibrosis improved; lung function either improved or remained stable. Definite conclusions regarding the clinical efficacy of RTX in SSc cannot be drawn but further exploration with a multicenter, randomized study is warranted.