B-cells in systemic sclerosis: emerging evidence from genetics to phenotypes. Issue 6 (November 2015)

• Record Type: Journal Article
• Authors: Wu, Minghua; Mohan, Chandra
• Is Part Of: Current opinion in rheumatology. Volume 27:Issue 6(2015:Nov.)
• Publication Date: 2015-11
• Subjects: antibodies -- autoimmunity -- cytokines -- fibrosis -- systemic sclerosis; Rheumatology -- Periodicals; Arthritis -- Periodicals; Review Literature -- Periodicals; Rheumatic Diseases -- Periodicals; 616.723
• DOI: 10.1097/BOR.0000000000000215
• Languages: English
• ISSNs: 1040-8711

Abstract

Abstract : 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)-β1, IL-1β 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.