Innate Immunity in Systemic Sclerosis Fibrosis: Recent Advances. Review uri icon

Overview

abstract

  • Systemic sclerosis (SSc) is a heterogeneous autoimmune disease characterized by three interconnected hallmarks (i) vasculopathy, (ii) aberrant immune activation, and (iii) fibroblast dysfunction leading to extracellular matrix deposition and fibrosis. Blocking or reversing the fibrotic process associated with this devastating disease is still an unmet clinical need. Although various components of innate immunity, including macrophages and type I interferon, have long been implicated in SSc, the precise mechanisms that regulate the global innate immune contribution to SSc pathogenesis remain poorly understood. Recent studies have identified new innate immune players, such as pathogen-recognition receptors, platelet-derived danger-associated molecular patterns, innate lymphoid cells, and plasmacytoid dendritic cells in the pathophysiology of SSc, including vasculopathy and fibrosis. In this review, we describe the evidence demonstrating the importance of innate immune processes during SSc development with particular emphasis on their role in the initiation of pathology. We also discuss potential therapeutic options to modulate innate immune cells or signaling in SSc that are emerging from these recent advances.

authors

  • Laurent, Paoline
  • Sisirak, Vanja
  • Lazaro, Estibaliz
  • Richez, Christophe
  • Duffau, Pierre
  • Blanco, Patrick
  • Truchetet, Marie-Elise
  • Contin-Bordes, Cécile

publication date

  • July 23, 2018

Identity

PubMed Central ID

  • PMC6064727

Scopus Document Identifier

  • 84919866420

Digital Object Identifier (DOI)

  • 10.1016/j.jaci.2014.05.011

PubMed ID

  • 30083163

Additional Document Info

volume

  • 9