Unraveling SSc Pathophysiology; The Myofibroblast.
Front Immunol
; 9: 2452, 2018.
Article
in En
| MEDLINE
| ID: mdl-30483246
ABSTRACT
Systemic sclerosis (SSc) is a severe auto-immune disease, characterized by vasculopathy and fibrosis of connective tissues. SSc has a high morbidity and mortality and unfortunately no disease modifying therapy is currently available. A key cell in the pathophysiology of SSc is the myofibroblast. Myofibroblasts are fibroblasts with contractile properties that produce a large amount of pro-fibrotic extracellular matrix molecules such as collagen type I. In this narrative review we will discuss the presence, formation, and role of myofibroblasts in SSc, and how these processes are stimulated and mediated by cells of the (innate) immune system such as mast cells and T helper 2 lymphocytes. Furthermore, current novel therapeutic approaches to target myofibroblasts will be highlighted for future perspective.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Scleroderma, Systemic
/
Myofibroblasts
Limits:
Humans
Language:
En
Journal:
Front Immunol
Year:
2018
Document type:
Article
Affiliation country:
Países Bajos