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Review

What have we learned from clinical trials in primary Sjögren's syndrome about pathogenesis?

Cees GM Kallenberg1*, Arjan Vissink2, Frans GM Kroese1, Wayel H Abdulahad1 and Hendrika Bootsma1

Author Affiliations

1 Department of Rheumatology and Clinical Immunology, AA21, University Medical Center Groningen, University of Groningen, P.O. Box 30.001, 9700 RB Groningen, The Netherlands

2 Department of Oral and Maxillofacial Surgery, University Medical Center Groningen, University of Groningen, 9700 RB Groningen, The Netherlands

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Arthritis Research & Therapy 2011, 13:205  doi:10.1186/ar3234

Published: 28 February 2011

Abstract

In vitro and in vivo experimental data have pointed to new immunopathogenic mechanisms in primary Sjögren's syndrome (pSS). The availability of targeted treatment modalities has opened new ways to selectively target these mechanistic pathways in vivo. This has taught us that the role of proinflammatory cytokines, in particular TNFα, is not crucial in the immunopathogenesis of pSS. B cells appear to play a major role, as depletion of B cells leads to restoration of salivary flow and is efficacious for treatment of extraglandular manifestations and mucosa-associated lymphoid tissue lymphoma. B cells also orchestrate T-cell infiltration and ductal epithelial dearrangement in the salivary glands. Gene profiling of salivary gland tissue in relation to B-cell depletion confirms that the axis of IFNα, B-cell activating factor, B-cell activation, proliferation and survival constitutes a major pathogenic route in pSS.