Journal
CURRENT OPINION IN RHEUMATOLOGY
Volume 27, Issue 6, Pages 537-541Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/BOR.0000000000000215
Keywords
antibodies; autoimmunity; cytokines; fibrosis; systemic sclerosis
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Funding
- Scleroderma Foundation
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Purpose of review This review aims to summarize current evidence for a role of B-cells in the pathogenesis of systemic sclerosis (SSc) from genetics to phenotypes, with an emphasis on recent insights. Recent findings Multiple genomic analyses have associated several B-cell signalling genes with SSc. Moreover, interesting B-cell subset alterations and activation/memory marker changes have also been documented in SSc. Co-cultures of blood B-cells with dermal fibroblasts isolated from SSc patients demonstrated the induction of collagen, interleukin (IL)-6, transforming growth factor (TGF)-beta 1, IL-1 beta and chemokine (c-c motif) ligand 2 (CCL2) in the fibroblasts, following potential B-cell cues delivered to the fibroblasts. Plasma cell gene signatures were elevated in SSc patients' blood, and highly correlated with collagen gene expression. Finally, anti-CD20 B-cell depletion therapy not only improved skin disease but also preserved interstitial lung disease in early diffuse cutaneous disease. Summary Thus, there is resounding evidence that B-cells play a pivotal role in pathogenesis of SSc. However, the molecular pathways through which B-cells may direct fibroblast function, SSc disease development and progression remain unclear, and warrant further study.
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