Molecular and cellular basis of scleroderma

J Mol Med (Berl). 2014 Sep;92(9):913-24. doi: 10.1007/s00109-014-1190-x. Epub 2014 Jul 18.

Abstract

Systemic sclerosis (scleroderma) is a chronic inflammatory disease that leads to fibrosis of the skin and involved internal organs. No efficient therapy is currently available. This review summarizes recent progress made in basic as well as clinical science and concludes with a concept that therapy targeting fibrosis in scleroderma needs to take into account the global microenvironment in the skin with its diverse cellular players interacting with a complex extracellular matrix environment and matrix-associated growth factors.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Extracellular Matrix / genetics
  • Extracellular Matrix / immunology
  • Extracellular Matrix / pathology*
  • Fibroblasts / immunology
  • Fibroblasts / metabolism
  • Fibroblasts / pathology*
  • Fibrosis
  • Humans
  • Inflammation / complications
  • Inflammation / immunology
  • Inflammation / pathology
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / genetics
  • Scleroderma, Systemic / immunology
  • Scleroderma, Systemic / pathology*
  • Signal Transduction
  • Skin / blood supply
  • Skin / immunology
  • Skin / metabolism
  • Skin / pathology*