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The most recent version of this article was published on 1 July 2005

Ann Rheum Dis. Published Online First: 25 November 2004. doi:10.1136/ard.2004.030049
Copyright © 2004 BMJ Publishing Group Ltd & European League Against Rheumatism.

Concise Report

X-linked agammaglobulinemia and rheumatoid arthritis

Gust Verbruggen 1, Sofie De Backer 2, Dieter Deforce 2, Pieter Demetter 2, Claude Cuvelier 2, Eric Veys 2 and Dirk Elewaut 2*

1 University of Gent, Belgium
2 University of Gent, Belgium

* To whom correspondence should be addressed. E-mail: dirk.elewaut{at}ugent.be.

Accepted 21 November 2004


Abstract

The pathogenic role of B cells in rheumatoid arthritis (RA) has recently gained much interest by the marked clinical responses of anti-CD20 therapy in RA. We describe a patient with X-linked agammaglobulinemia (XLA) who presented with an erosive symmetrical polyarthritis with histological features of RA including formation of a destructive pannus. Furthermore, the patient also developed subcutaneous nodules that were histologically undistinguishable from rheumatoid nodules. Surprisingly, lymphocytic infiltrates in both synovium and nodule consisted almost exclusively of CD8+ T cells. Although some peculiar B cell subsets have been described in XLA patients, no B cell subsets could be demonstrated in synovial tissue or the subcutaneous nodule. This case illustrates that classical RA can develop in the absence of mature B cells

Keywords: B cells, X-linked agammaglobulinemia, rheumatoid arthritis


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