Introduction: The etiopathogenesis of systemic sclerosis-an integrated overview

Carol Feghali-Bostwick*, John Varga

*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Systemic sclerosis (SSc) is a heterogeneous chronic multisystem disease of unknown etiology. It has global distribution and, like other connective diseases, a strong female predominance. The onset of SSc shows age-dependence and modest familial clustering, but no compelling geographic or temporal clustering. Multiple genes are implicated in SSc susceptibility, and the genetic architecture, dominated by the HLA locus, shows considerable overlap with other autoimmune diseases. The variable disease course, its high burden of mortality and morbidity, and lack of effective therapies make SSc a top research priority in the field of rheumatic diseases. A bird’s-eye view of SSc suggests the following paradigm for disease pathogenesis, supported by current data in the chapters that follow: initial injury in an individual with a permissive genetic background triggers complex and sustained interactions among immune and vascular cells in a variety of tissues. Oxidative stress ensues, and a range of soluble and matrix-associated signals are generated that trigger and maintain mesenchymal cell activation and survival, leading to matrix accumulation and culminating in progressive multi-organ fibrosis.

Original languageEnglish (US)
Title of host publicationScleroderma
Subtitle of host publicationFrom Pathogenesis to Comprehensive Management
PublisherSpringer International Publishing
Pages133-139
Number of pages7
ISBN (Electronic)9783319314075
ISBN (Print)9783319314051
DOIs
StatePublished - Jan 1 2016

Keywords

  • Autoimmunity
  • Fibrosis
  • Genetics
  • Inflammation
  • Innate immunity
  • Microvasculopathy
  • Myofibroblast
  • Pericyte

ASJC Scopus subject areas

  • Medicine(all)

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