Immunopathogenesis of Juvenile Systemic Sclerosis. Review uri icon

Overview

abstract

  • Juvenile-onset systemic sclerosis (jSSc) is a rare and severe autoimmune disease with associated life-threatening organ inflammation and evidence of fibrosis. The organ manifestations of jSSc resemble adult SSc, but with better outcomes and survival. The etiology of jSSc appears to reflect adult-onset SSc, with similar inflammatory mediators and autoantibodies, but with a significant population of children with uncharacterized anti-nuclear antibodies. The genetics of patients with jSSc differ from women with SSc, resembling instead the genes of adult males with SSc, with additional HLA genes uniquely associated with childhood-onset disease. Current treatments are aimed at inhibiting the inflammatory aspect of disease, but important mechanisms of fibrosis regulated by dermal white adipose tissue dendritic cells may provide an avenue for targeting and potentially reversing the fibrotic stage.

publication date

  • June 25, 2019

Research

keywords

  • Antibodies, Antinuclear
  • Dendritic Cells
  • Dermis
  • Scleroderma, Systemic
  • Subcutaneous Fat

Identity

PubMed Central ID

  • PMC6603145

Scopus Document Identifier

  • 85069042500

Digital Object Identifier (DOI)

  • 10.1016/j.bbmt.2018.06.025

PubMed ID

  • 31293569

Additional Document Info

volume

  • 10