Systemic sclerosis



Systemic sclerosis (SSc) is a systemic connective tissue disease. Characteristics of systemic sclerosis include essential vasomotor disturbances; fibrosis; subsequent atrophy of the skin (see the image below), subcutaneous tissue, muscles, and internal organs (eg, alimentary tract, lungs, heart, kidney, CNS); and immunologic disturbances accompany these findings.

 
CANDIDATE APPLICATION


State of the art. Autologous fat graft and adipose tissue-derived stromal vascular fraction injection for hand therapy in systemic sclerosis patients.
 
 Curr Res Transl Med. 2016 Jan-Mar;64(1):35-42. doi: 10.1016/j.retram.2016.01.006. Epub 2016 Mar 2.Authors: Guillaume-Jugnot P, Daumas A, Magalon J, Sautereau N, Veran J, Magalon G, Sabatier F, Granel B.

Abstract
 

Systemic sclerosis is an autoimmune disease characterized by sclerosis (hardening) of the skin and deep viscera associated with microvascular functional and structural alteration, which leads to chronic ischemia. In the hands of patients, ischemic and fibrotic damages lead to both pain and functional impairment. Hand disability creates a large burden in professional and daily activities, with social and psychological consequences. Currently, the proposed therapeutic options for hands rely mainly on hygienic measures, vasodilatator drugs and physiotherapy, but have many constraints and limited effects. Developing an innovative therapeutic approach is crucial to reduce symptoms and improve the quality of life. The discovery of adult stem cells from adipose tissue has increased the interest to use adipose tissue in plastic and regenerative surgery. Prepared as freshly isolated cells for immediate autologous transplantation, adipose tissue-derived stem cell therapy has emerged as a therapeutic alternative for the regeneration and repair of damaged tissues. We aim to update literature in the interest of autologous fat graft or adipose derived from stromal vascular fraction cell-based therapy for the hands of patients who suffer from systemic sclerosis.

 
 Adipose-Derived Mesenchymal Stem Cells in Autoimmune Disorders: State of the Art and Perspectives for Systemic Sclerosis.
 

Clin Rev Allergy Immunol. 2017 Apr;52(2):234-259. doi: 10.1007/s12016-016-8552-9.

Authors: Maria AT, Maumus M, Le Quellec A, Jorgensen C, Noël D, Guilpain P.

Abstract
 

Mesenchymal stromal/stem cells (MSC) are non-hematopoietic multipotent progenitor cells, first described in bone marrow in the middle of last century. Since then, MSC have been the objects of a myriad of publications, progressively increasing our knowledge on their potentialities and bringing high expectancies for their regenerative properties. During the same period, numerous tissues, such as adipose tissue, placenta, or umbilical cord, have been used as alternative sources of MSC in comparison with bone marrow. In particular, considering the accessibility and ease to harvest fat tissue, adipose-derived MSC have gained interest above bone marrow-derived MSC. More recently, the discovery of MSC immunomodulatory properties made MSC-based therapy progressively slip from the field of regenerative medicine to the one of autoimmunity. Indeed, in this group of disorders caused by aberrant activation of the immune system resulting in loss of self-tolerance and auto-reactivity, conventional immunosuppressant may be harmful. One advantage of MSC-based therapy would lie in their immune plasticity, resulting in space and time-limited immunosuppression. More specifically, among autoimmune disorders, systemic sclerosis appears as a peculiar multifaceted disease, in which autoimmune phenomena coexist with vascular abnormalities and multi-visceral fibrosis. Considering the pleiotropic effects of MSC, displaying immunomodulatory, angiogenic and antifibrotic capabilities, MSC-based therapy could counteract the three main pathogenic axes of systemic sclerosis and might thus represent a complete breakthrough in this intractable disease with unmet medical need. In this article, while reviewing most recent literature on MSC biology, we itemize their current applications in the field of autoimmunity and shed light onto the potential use of adipose-derived MSC as an innovative strategy to cure systemic sclerosis.

“More research is needed with larger numbers of patients who are randomized to receive either the or an approved therapy, but it’s very exciting to see that this treatment may be so superior to a current treatment for people with severe MS that is not responding well to standard treatments,” Mancardi said.

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