Mesenchymal stromal cells for systemic sclerosis treatment

Systemic sclerosis (SSc) is a rare chronic autoimmune disease characterized by vasculopathy, dysregulation of innate and adaptive immune responses, and progressive fibrosis. SSc remains an orphan disease, with high morbity and mortality in SSc patients. The mesenchymal stromal cells (MSC) demonstrat...

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Published inAutoimmunity reviews Vol. 20; no. 3; p. 102755
Main Authors Farge, Dominique, Loisel, Séverine, Lansiaux, Pauline, Tarte, Karin
Format Journal Article
LanguageEnglish
Published Netherlands Elsevier B.V 01.03.2021
Elsevier
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Summary:Systemic sclerosis (SSc) is a rare chronic autoimmune disease characterized by vasculopathy, dysregulation of innate and adaptive immune responses, and progressive fibrosis. SSc remains an orphan disease, with high morbity and mortality in SSc patients. The mesenchymal stromal cells (MSC) demonstrate in vitro and in vivo pro-angiogenic, immuno-suppressive, and anti-fibrotic properties and appear as a promising stem cell therapy type, that may target the key pathological features of SSc disease. This review aims to summarize acquired knowledge in the field of :1) MSC definition and in vitro and in vivo functional properties, which vary according to the donor type (allogeneic or autologous), the tissue sources (bone marrow, adipose tissue or umbilical cord) or inflammatory micro-environment in the recipient; 2) preclinical studies in various SSc animal models , which showed reduction in skin and lung fibrosis after MSC infusion; 3) first clinical trials in human, with safety and early efficacy results reported in SSc patients or currently tested in several ongoing clinical trials. •Systemic sclerosis (SSc) is a rare chronic autoimmune orphan disease.•SSc pathogenesis associates vasculopathy, immune dysregulation and fibrosis.•Mesenchymal stromal cells (MSC) properties can target the SSc pathogenic triad.•In vitro and in vivo MSC properties vary with donor sources and local inflammation.•MSC appear as a new promising stem cell therapy for SSc patients.
ISSN:1568-9972
1568-9972
1873-0183
DOI:10.1016/j.autrev.2021.102755