New developments in the pathogenesis of systemic sclerosis.

Abstract:

:Systemic sclerosis (SSc) is characterized by extensive fibrosis, vasculopathy and activation of the immune system. Fibrosis can be caused by profibrotic cytokines, such as transforming growth factor-beta (TGFbeta), interleukin-4 (IL-4), platelet-derived growth factor (PDGF), and connective tissue growth factor. Vasculopathy can be caused by TGFbeta, PDGF, while paucity of vessels in skin lesions can be attributed to anti-endothelial cell autoantibodies. Recent studies have suggested that the activation of the immune system is of paramount importance in the pathogenesis of SSc. T Cells are activated by antigen, infiltrate early the skin lesions in SSc, and produce the profibrotic cytokine IL-4. They are also required for autoantibody production. B cells may contribute to fibrosis, as deficiency of CD19, a B cell transduction molecule, results in decreased fibrosis in animal models of fibrosis. These new developments have direct impact on the treatment of SSc. Medications directed against immune cells or harmful soluble factors in small trials in SSc are encouraging.

journal_name

Autoimmunity

journal_title

Autoimmunity

authors

Sakkas LI

doi

10.1080/16066350500095415

subject

Has Abstract

pub_date

2005-03-01 00:00:00

pages

113-6

issue

2

eissn

0891-6934

issn

1607-842X

pii

R7N4MG839K3763L6

journal_volume

38

pub_type

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