Frontiers in Immunology (Jul 2018)

Innate Immunity in Systemic Sclerosis Fibrosis: Recent Advances

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

DOI
https://doi.org/10.3389/fimmu.2018.01702
Journal volume & issue
Vol. 9

Abstract

Read online

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.

Keywords