Unraveling SSc Pathophysiology; The Myofibroblast
Publication year
2018Source
Frontiers in Immunology, 9, (2018), article 2452ISSN
Publication type
Article / Letter to editor

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Organization
Rheumatology
Journal title
Frontiers in Immunology
Volume
vol. 9
Subject
Radboudumc 5: Inflammatory diseases RIHS: Radboud Institute for Health Sciences; Radboudumc 5: Inflammatory diseases RIMLS: Radboud Institute for Molecular Life SciencesAbstract
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.
This item appears in the following Collection(s)
- Academic publications [227613]
- Electronic publications [107274]
- Faculty of Medical Sciences [86193]
- Open Access publications [76400]
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