Innate Immunity in Systemic Sclerosis

Christopher Dowson, Nathan Simpson, Laura Duffy, Steven O'Reilly

Research output: Contribution to journalArticlepeer-review

33 Citations (Scopus)

Abstract

Purpose of Review Systemic sclerosis (SSc) is a heterogeneous autoimmune disease which has defined three hallmarks: Small vessel vasculopathy, production of autoantibodies and fibroblast dysfunction. The exact aetiology of the disease remains unknown, due to the complex nature of the cellular signalling pathways involved. However, there is strong and consistent evidence that the innate system, in particular toll-like receptor signalling, is contributing to the progression and perhaps onset of systemic sclerosis. In light of this evidence, this review examines the role of innate immunity in systemic sclerosis and where appropriate suggests avenues for therapeutic modulation in SSc. Recent Findings Multiple lines of evidence suggest that Toll-like receptors (TLRs) are dysregulated and emerging evidence suggests that many endogenous ligands are also elevated in the disease leading to ‘sterile inflammation’ and ultimately the induction of fibrosis. Currently, no effective therapy exists and exploiting the innate immune system perturbation may be one possible avenue.
Original languageEnglish
Article number2
JournalCurrent Rheumatology Reports
Volume19
Issue number1
Early online date23 Jan 2017
DOIs
Publication statusPublished - Jan 2017

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