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

Cees G. M. Kallenberg*, Arjan Vissink, Frans G. M. Kroese, Wayel H. Abdulahad, Hendrika Bootsma

*Corresponding author for this work

    Research output: Contribution to journalReview articlepeer-review

    29 Citations (Scopus)
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    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.

    Original languageEnglish
    Article number205
    Number of pages8
    JournalArthritis Research and Therapy
    Volume13
    Issue number1
    DOIs
    Publication statusPublished - 28-Feb-2011

    Keywords

    • NECROSIS-FACTOR-ALPHA
    • SYSTEMIC-LUPUS-ERYTHEMATOSUS
    • LABIAL SALIVARY-GLANDS
    • CELL-ACTIVATING FACTOR
    • INTERFERON-ALPHA
    • RITUXIMAB TREATMENT
    • DOUBLE-BLIND
    • EPITHELIAL-CELLS
    • GENE-EXPRESSION
    • FAMILY BAFF

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