Research Topic

Key Players in Systemic Sclerosis: The Immune System and Beyond

About this Research Topic

Systemic sclerosis (SSc, also called scleroderma) is a complex rare multifaceted disease characterized by three main mechanisms: fibrosis, vascular involvement and autoimmunity. Fibrosis primarily affects the skin dermis. However, the lungs, heart, and/or digestive tract may also be involved. Fibrosis is the consequence of an excessive accumulation of collagen promoted by abnormal fibroblast activation. Vascular impairment in SSc leads to the development of Raynaud’s phenomenon, telangiectasia, digital ulcers, pulmonary arterial hypertension and/or vascular renal crisis. These features render SSc a very peculiar autoimmune disorder. Autoimmunity in SSc is mediated via immune cell activation and via production of autoantibodies against three main target autoantigens, namely Topoisomerase 1, CENP-A/B or RNA-polymerase III. SSc has a poor prognosis globally, and its treatment is mainly palliative and based on symptomatic therapies, vasodilators and immunosuppressants. Therefore, advances in our understanding of the crucial actors and mechanisms that underlie this disease are essential for improving therapy and patient outcomes.

A myriad of questions regarding SSc pathogenesis still remain unresolved. The lack of truly effective treatments prompts research teams to improve the understanding of this disease and to develop innovative therapeutic approaches. The mechanisms leading to fibroblast activation and endothelial impairment are likely dependent on environmental, genetic and epigenetic factors, with a special role of endogenous and/or exogenous oxidative stress. Recent studies focusing on the link between cancer and SSc, demonstrated that the immune response could be directed against malignant targets. Metabolic pathways (such as those implicating PPAR-gamma) are also likely important for SSc development and could represent novel therapeutic targets. Mesenchymal stem cells could be causative actors or –conversely- therapeutic agents for SSc, and their potentially double-sided status in SSc is currently under intense investigation. Several pharmacological approaches are also under development to counteract fibrosis and vascular impairment in SSc.

In this Research Topic, we aim to focus on various cellular, molecular and environmental actors involved in SSc, in relationship with the immune system, and on innovative therapies for SSc in order to advance our understanding and treatment of this disease. By exploring the most recent advances and discoveries in this field, this Research Topic aims to transition our understanding of SSc pathogenesis from its old conception to a modern view. We welcome the submission of Original Research, Review, Mini-Review, Perspective, Case Report, Clinical Trial and General Commentary articles presenting studies on animal models of SSc and/or human SSc patients. The topics to be covered in this Research Topic include:

1. The relationship between SSc clinical phenotype and autoantibodies.
2. Cancer immunosurveillance and immunoediting in SSc.
3. Immunomodulatory functions of stem cells in SSc.
4. Immune system and epigenetics in SSc.
5. Environmental factors, oxidative stress and immune system in SSc.
6. Metabolic pathways and immune system in SSc.
7. Breakdown of immune tolerance in SSc.
8. Immune system as a target for pharmacological and cell therapies (including MSC-based therapies and HSC-based therapies).


Keywords: Systemic sclerosis, Endothelium, Fibrosis, Autoimmunity, Therapy


Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.

Systemic sclerosis (SSc, also called scleroderma) is a complex rare multifaceted disease characterized by three main mechanisms: fibrosis, vascular involvement and autoimmunity. Fibrosis primarily affects the skin dermis. However, the lungs, heart, and/or digestive tract may also be involved. Fibrosis is the consequence of an excessive accumulation of collagen promoted by abnormal fibroblast activation. Vascular impairment in SSc leads to the development of Raynaud’s phenomenon, telangiectasia, digital ulcers, pulmonary arterial hypertension and/or vascular renal crisis. These features render SSc a very peculiar autoimmune disorder. Autoimmunity in SSc is mediated via immune cell activation and via production of autoantibodies against three main target autoantigens, namely Topoisomerase 1, CENP-A/B or RNA-polymerase III. SSc has a poor prognosis globally, and its treatment is mainly palliative and based on symptomatic therapies, vasodilators and immunosuppressants. Therefore, advances in our understanding of the crucial actors and mechanisms that underlie this disease are essential for improving therapy and patient outcomes.

A myriad of questions regarding SSc pathogenesis still remain unresolved. The lack of truly effective treatments prompts research teams to improve the understanding of this disease and to develop innovative therapeutic approaches. The mechanisms leading to fibroblast activation and endothelial impairment are likely dependent on environmental, genetic and epigenetic factors, with a special role of endogenous and/or exogenous oxidative stress. Recent studies focusing on the link between cancer and SSc, demonstrated that the immune response could be directed against malignant targets. Metabolic pathways (such as those implicating PPAR-gamma) are also likely important for SSc development and could represent novel therapeutic targets. Mesenchymal stem cells could be causative actors or –conversely- therapeutic agents for SSc, and their potentially double-sided status in SSc is currently under intense investigation. Several pharmacological approaches are also under development to counteract fibrosis and vascular impairment in SSc.

In this Research Topic, we aim to focus on various cellular, molecular and environmental actors involved in SSc, in relationship with the immune system, and on innovative therapies for SSc in order to advance our understanding and treatment of this disease. By exploring the most recent advances and discoveries in this field, this Research Topic aims to transition our understanding of SSc pathogenesis from its old conception to a modern view. We welcome the submission of Original Research, Review, Mini-Review, Perspective, Case Report, Clinical Trial and General Commentary articles presenting studies on animal models of SSc and/or human SSc patients. The topics to be covered in this Research Topic include:

1. The relationship between SSc clinical phenotype and autoantibodies.
2. Cancer immunosurveillance and immunoediting in SSc.
3. Immunomodulatory functions of stem cells in SSc.
4. Immune system and epigenetics in SSc.
5. Environmental factors, oxidative stress and immune system in SSc.
6. Metabolic pathways and immune system in SSc.
7. Breakdown of immune tolerance in SSc.
8. Immune system as a target for pharmacological and cell therapies (including MSC-based therapies and HSC-based therapies).


Keywords: Systemic sclerosis, Endothelium, Fibrosis, Autoimmunity, Therapy


Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.

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Submission Deadlines

31 January 2018 Abstract
30 April 2018 Manuscript

Participating Journals

Manuscripts can be submitted to this Research Topic via the following journals:

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Topic Editors

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Submission Deadlines

31 January 2018 Abstract
30 April 2018 Manuscript

Participating Journals

Manuscripts can be submitted to this Research Topic via the following journals:

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