Consistent stimulating effects on extracellular matrix production Regulation of fibroblast proliferation
CTGF
A active stimulant of collagen and fibronectin production Increase the transcription of type I collagen
PDGF
Stimulate the production of extracellular matrix components Stimulate autocrine production of a profibrotic chemokine, MCP-1
OSM
Stimulates proliferation of fibroblasts Production of collagen and glycosaminoglycan
Interleukin-4
Stimulate fibroblast proliferation Produce collagen, chemotaxis, expression of adhesion molecules ICAM-1 and VCAM-1
IFN-γ
Inhibit fibroblast proliferation and production of collagen
MCP-1
Upregulates production of collagen through autocrine TGF- b
PARC
Stimulate collagen mRNA and protein production through activation of transcription factors Sp1 and c-Myb
Potential cellular sources of the profibrotic cytokines and chemokines in scleroderma lung disease include alternatively activated macrophages, activated CD8+T cells,
eosinophils, mast cells, epithelial cells and fibroblasts themselves.
Table 2: Proinflammatory and profibrotic cytokines.
