Pathophysiology of fibrosis in systemic sclerosis

Maria Trojanowska*, John Varga

*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Scopus citations

Abstract

Fibrogenesis is a multistage pathological process leading to scarring of virtually any organ. Fibrosis is characterized by disruption of normal tissue architecture and its replacement with stiff collagen-rich connective tissue. The process results in progressive functional impairment, culminating in organ failure. Fibrosis is the hallmark of scleroderma, as well as a large and heterogeneous collection of human diseases. In these conditions, fibrosis represents the end result of a complex series of vascular and immune-mediated responses to chronic or recurrent injury in a genetically predisposed individual. As illustrated in Fig. 18.1, injured activated vascular, epithelial, and immune cells generate soluble mediators, functional autoantibodies, and reactive oxygen species (ROS) that serve as cues for mesenchymal cells to induce their sustained activation, differentiation, and survival, leading to excessive matrix deposition, tissue stiffness, and, ultimately, fibrosis. While fibrosis is potentially reversible, effective therapies to prevent or repair fibrosis are not yet available.

Original languageEnglish (US)
Title of host publicationScleroderma
Subtitle of host publicationFrom Pathogenesis to Comprehensive Management
PublisherSpringer International Publishing
Pages261-280
Number of pages20
ISBN (Electronic)9783319314075
ISBN (Print)9783319314051
DOIs
StatePublished - Jan 1 2016

Keywords

  • Collagen
  • Cytokine
  • Egr-1
  • Fibroblast
  • Fibrosis
  • Matrix
  • Myofibroblast
  • Pericyte
  • Smad
  • TGF-β

ASJC Scopus subject areas

  • Medicine(all)

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