Autoantibodies as markers and possible mediators of scleroderma pathogenesis

Kimberly Doering Maurer, Antony Rosen

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Scleroderma or systemic sclerosis (SSc) is a systemic autoimmune disease characterized by a distinct, proliferative vasculopathy associated with skin thickening and fibrosis within internal organs and is associated with a unique autoimmune response. Within scleroderma, specific immune responses are strikingly associated with distinct clinical features. These autoantibody-defined clinical subgroups have distinct phenotypic and biological behavior, suggesting that the selection of specific immune targets has pathogenic consequences. This chapter focuses on the specificity and dynamics of these immune responses and the insights that they provide into disease mechanism, including as markers of key pathogenic events that initiate specific immune responses (e.g., mutation of scleroderma autoantigens in cancers), as well as potential roles as active mediators of damage of specific tissues. An understanding of the mechanisms underlying this extraordinary specificity may provide novel therapeutic targets or disease monitoring and management strategies.

Original languageEnglish (US)
Title of host publicationScleroderma: From Pathogenesis to Comprehensive Management
PublisherSpringer International Publishing
Pages197-205
Number of pages9
ISBN (Electronic)9783319314075
ISBN (Print)9783319314051
DOIs
StatePublished - Jan 1 2016

Keywords

  • Anti-endothelial cell antibodies
  • Anti-fibroblast antibodies
  • Autoantibodies
  • Autoantigens
  • B cells
  • Cytotoxic T lymphocytes
  • Extracellular matrix
  • Granzyme B
  • Humoral immunity
  • Immune complexes
  • Interferon-α
  • Malignancy
  • Pathogenesis
  • PDGFR antibodies
  • Plasmacytoid dendritic cells
  • RNA polymerase III autoantibodies
  • Topoisomerase-1 autoantibodies

ASJC Scopus subject areas

  • General Medicine

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