Fibrosis and Immune Dysregulation in Systemic Sclerosis

2018 ◽  
pp. 25-60
Author(s):  
Yahya Argobi ◽  
Gideon P. Smith
Keyword(s):  
Systemic Sclerosis ◽  
Immune Dysregulation

scholarly journals The Role of PPAR Gamma in Systemic Sclerosis

PPAR Research ◽  
2015 ◽  
Vol 2015 ◽  
pp. 1-12 ◽  
Author(s):  
Andréa Tavares Dantas ◽  
Michelly Cristiny Pereira ◽  
Moacyr Jesus Barreto de Melo Rego ◽  
Laurindo Ferreira da Rocha ◽  
Ivan da Rocha Pitta ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Lung Fibrosis ◽  
Ppar Gamma ◽  
Immune Dysregulation ◽  
Unknown Etiology ◽  
Internal Organs ◽  
Medical Need

Fibrosis is recognized as an important feature of many chronic diseases, such as systemic sclerosis (SSc), an autoimmune disease of unknown etiology, characterized by immune dysregulation and vascular injury, followed by progressive fibrosis affecting the skin and multiple internal organs. SSc has a poor prognosis because no therapy has been shown to reverse or arrest the progression of fibrosis, representing a major unmet medical need. Recently, antifibrotic effects of PPARγligands have been studiedin vitroandin vivoand some theories have emerged leading to new insights. Aberrant PPARγfunction seems to be implicated in pathological fibrosis in the skin and lungs. This antifibrotic effect is mainly related to the inhibition of TGF-β/Smad signal transduction but other pathways can be involved. This review focused on recent studies that identified PPARγas an important novel pathway with critical roles in regulating connective tissue homeostasis, with emphasis on skin and lung fibrosis and its role on systemic sclerosis.

Pathogenesis of systemic sclerosis: recent insights of molecular and cellular mechanisms and therapeutic opportunities

2017 ◽  
Vol 2 (3) ◽  
pp. 137-152 ◽  
Author(s):  
John Varga ◽  
Maria Trojanowska ◽  
Masataka Kuwana
Keyword(s):  
Systemic Sclerosis ◽  
Complex Disease ◽  
Repair Process ◽  
Immune Dysregulation ◽  
Lipid Mediators ◽  
Oxygen Species ◽  
Cellular Mechanisms ◽  
Internal Organs ◽  
Matrix Deposition ◽  
Pathogenic Process

Systemic sclerosis (SSc) is a complex disease characterized by early microvascular abnormalities, immune dysregulation and chronic inflammation, and subsequent fibrosis of the skin and internal organs. Excessive fibrosis, distinguishing hallmark of SSc, is the end result of a complex series of interlinked vascular injury and immune activation, and represents a maladaptive repair process. Activated vascular, epithelial, and immune cells generate pro-fibrotic cytokines, chemokines, growth factors, lipid mediators, autoantibodies, and reactive oxygen species. These paracrine and autocrine cues in turn induce activation, differentiation, and survival of mesenchymal cells, ensuing tissue fibrosis through increased collagen synthesis, matrix deposition, tissue rigidity and remodeling, and vascular rarefaction. This review features recent insights of the pathogenic process of SSc, highlighting three major characteristics of SSc, microvasculopathy, excessive fibrosis, and immune dysregulation, and sheds new light on the understanding of molecular and cellular mechanisms contributing to the pathogenesis of SSc and providing novel avenues for targeted therapies.

scholarly journals Unfolding the pathogenesis of systemic sclerosis through epigenomics

STEMedicine ◽  
2020 ◽  
Vol 1 (1) ◽  
pp. e5
Author(s):  
Xiuzhi Jia ◽  
Hao Cheng ◽  
Ying Xiao
Keyword(s):  
Dna Methylation ◽  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Environmental Stress ◽  
Histone Modification ◽  
Immune Dysregulation ◽  
Epigenetic Mechanisms ◽  
Internal Organs ◽  
Current Information ◽  
Epigenetic Alteration

As a group of autoimmune diseases, systemic sclerosis (scleroderma, SSc) is characterized by immune dysregulation, micro-vessels dominant obliteration, and the final fibrosis of the skin and or internal organs. Although the precise mechanisms are still unknown, increasing data shows that epigenetic mechanisms, such as DNA methylation, histone modification, and microRNA (miRNA), are strictly related to the pathogenesis of scleroderma. Epigenetic mechanisms, which can link genetics and environmental stress, represents a promising field in systemic sclerosis investigation. The objective of this review is, to sum up the current information about epigenetic alteration.

Fibrosis and immune dysregulation in systemic sclerosis

2011 ◽  
Vol 10 (5) ◽  
pp. 276-281 ◽  
Author(s):  
Carlo Chizzolini ◽  
Nicolò C. Brembilla ◽  
Elisa Montanari ◽  
Marie-Elise Truchetet
Keyword(s):  
Systemic Sclerosis ◽  
Immune Dysregulation

scholarly journals Epstein Barr Virus and Autoimmune Responses in Systemic Lupus Erythematosus

2021 ◽  
Vol 11 ◽  
Author(s):  
Neelakshi R. Jog ◽  
Judith A. James
Keyword(s):  
Multiple Sclerosis ◽  
Systemic Lupus Erythematosus ◽  
Systemic Sclerosis ◽  
Lupus Erythematosus ◽  
Epstein Barr Virus ◽  
Immune Dysregulation ◽  
Systemic Autoimmune Disease ◽  
Systemic Lupus ◽  
Barr Virus ◽  
Epstein Barr

Systemic lupus erythematosus (SLE) is a complex systemic autoimmune disease. Infections or infectious reactivation are potential triggers for initiation of autoimmunity and for SLE flares. Epstein-Barr virus (EBV) is gamma herpes virus that has been associated with several autoimmune diseases such as SLE, multiple sclerosis, Sjogren’s syndrome, and systemic sclerosis. In this review, we will discuss the recent advances regarding how EBV may contribute to immune dysregulation, and how these mechanisms may relate to SLE disease progression.

scholarly journals Treatment of Systemic Sclerosis Associated Interstitial Lung Disease

2020 ◽  
Vol 20 (02) ◽  
pp. 56-64
Author(s):  
Iris Yan Ki Tang ◽  
So Ho
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Immune Dysregulation ◽  
Connective Tissue Disorder ◽  
Major Contributor ◽  
Biological Therapies ◽  
Visceral Organ ◽  
Common Manifestation ◽  
Organ Fibrosis

Systemic sclerosis (SSc) is a systemic connective tissue disorder characterized by progressive skin and visceral organ fibrosis, vasculopathy, and immune dysregulation. Interstitial lung disease (ILD) is a common manifestation and major contributor to morbidity and mortality. Immunosuppression is usually indicated for extensive or progressive SSc-ILD. Recently, antifibrotic and biological therapies have been shown to be efficacious in treating SSc-ILD in various studies. In this article, we will descriptively review the latest evidence on the treatment of SSc-ILD.

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