The immunopathogenesis of fibrosis in systemic sclerosis.

Abstract:

:Systemic sclerosis (SSc) is an idiopathic systemic autoimmune disease. It is characterized by a triad of hallmarks: immune dysfunction, fibrosis and vasculopathy. Immune dysfunction in SSc is characterized by the activation and recruitment of immune cells and the production of autoantibodies and cytokines. How immune abnormalities link the fibrosis and vasculopathy in SSc is poorly understood. A plethora of immune cell types are implicated in the immunopathogenesis of SSc, including T cells, B cells, dendritic cells, mast cells and macrophages. How these different cell types interact to contribute to SSc is complicated, and can involve cell-to-cell interactions and communication via cytokines, including transforming growth factor (TGF)-β, interleukin (IL)-6 and IL-4. We will attempt to review significant and recent research demonstrating the importance of immune cell regulation in the immunopathogenesis of SSc with a particular focus on fibrosis.

journal_name

Clin Exp Immunol

authors

Brown M,O'Reilly S

doi

10.1111/cei.13238

subject

Has Abstract

pub_date

2019-03-01 00:00:00

pages

310-321

issue

3

eissn

0009-9104

issn

1365-2249

journal_volume

195

pub_type

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