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Front. Immunol. | doi: 10.3389/fimmu.2018.02452

Unraveling SSc pathophysiology; the myofibroblast

 Arjan Van Caam1*, Madelon Vonk1, Frank van den Hoogen1, Peter van Lent1 and Peter van der Kraan1
  • 1Radboud University Nijmegen Medical Centre, Netherlands

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.

Keywords: myofibroblast, Systemic scleroderma, Immune System, Treatm ent, cytokine

Received: 17 Jun 2018; Accepted: 04 Oct 2018.

Edited by:

Urs Christen, Goethe-Universität Frankfurt am Main, Germany

Reviewed by:

Grazyna Kwapiszewska, Ludwig Boltzmann Institute for Lung Vascular Research, Austria
DIMITRIOS P. BOGDANOS, University of Thessaly, Greece  

Copyright: © 2018 Van Caam, Vonk, van den Hoogen, van Lent and van der Kraan. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Dr. Arjan Van Caam, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands,