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Pathogenesis, Clinical Diagnosis, Treatments and Managements of Systemic Sclerosis
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Pathogenesis, Clinical Diagnosis, Treatments and Managements of Systemic Sclerosis

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Dermatology".

Deadline for manuscript submissions: closed (31 December 2020) | Viewed by 23374

Special Issue Editor

Prof. Dr. Shinichi Sato

E-Mail Website
Guest Editor
Department of Dermatology, University of Tokyo, Tokyo 113-8654, Japan
Interests: scleroderma; B lymphocytes; autoimmunity

Special Issue Information

Dear Colleagues,

Systemic sclerosis (SSc) is a connective tissue disorder characterized by fibrosis in the skin and internal organs. Although the pathogenesis of SSc remains unknown, collagen accumulation, vascular damage, and abnormal immune activation are major abnormalities. In particular, since immunosuppresants are effective for fibrosis in SSc, abnormal immune regulation is considered to be most important. However, understanding the pathogenesis from other perspectives, such as fibroblast and vascular endothelial cell abnormalities, is equally important. There are also many points to be solved clinically. For example, since interstitial lung disease associated with SSc does not progress in all patients, how do we identify the interstitial lung disease that will progress in the future? The purpose of this Special Issue is to understand the pathogenesis of SSc and to provide the latest knowledge on important clinical issues. Therefore, researchers in the field of SSc are encouraged to submit an original article or review to this Special Issue.

Prof. Dr. Shinichi Sato
Guest Editor

Manuscript Submission Information

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Keywords

  • immune activation
  • fibroblast activation
  • vascular abnormality
  • diagnosis
  • natural course
  • biomarker
  • therapy
  • clinical study
  • primary endpoint

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Published Papers (4 papers)

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Research

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15 pages, 3281 KiB  
Article
Implication of miR-126 and miR-139-5p in Plasmacytoid Dendritic Cell Dysregulation in Systemic Sclerosis
by Eleni Chouri, Maojie Wang, Maarten R. Hillen, Chiara Angiolilli, Sandra C. Silva-Cardoso, Catharina G. K. Wichers, Maarten van der Kroef, Cornelis P. J. Bekker, Marta Cossu, Lenny van Bon, Alsya J. Affandi, Tiago Carvalheiro, Aridaman Pandit, Joel A. G. van Roon, Lorenzo Beretta, Boudewijn M. T. Burgering, Timothy R. D. J. Radstake and Marzia Rossato
J. Clin. Med. 2021, 10(3), 491; https://doi.org/10.3390/jcm10030491 - 30 Jan 2021
Cited by 22 | Viewed by 3746
Abstract
Compelling evidence shows the involvement of plasmacytoid dendritic cells (pDCs) in systemic sclerosis (SSc) pathogenesis. This study investigated whether microRNAs (miRNAs) are involved in the dysregulation of pDCs in SSc patients already at early stages. RNA from circulating pDCs was isolated from two [...] Read more.
Compelling evidence shows the involvement of plasmacytoid dendritic cells (pDCs) in systemic sclerosis (SSc) pathogenesis. This study investigated whether microRNAs (miRNAs) are involved in the dysregulation of pDCs in SSc patients already at early stages. RNA from circulating pDCs was isolated from two independent cohorts of SSc patients with different disease phenotypes, and individuals with Raynaud’s phenomenon, for microRNA profiling and RNA-sequencing analysis. Proteomic analysis was exploited to identify novel direct miRNA targets at the protein level. Twelve and fifteen miRNAs were differentially expressed in at least one group of patients compared to healthy controls in discovery cohort I and II, respectively. Of note, miR-126 and miR-139-5p were upregulated in both preclinical and definite SSc patients and correlated with the expression of type I interferon (IFN)-responsive genes. Toll-like receptor 9 (TLR9) stimulation of healthy pDCs upregulated the expression of both miRNAs, similarly to what was observed in patients. The proteomic analysis identified USP24 as a novel target of miR-139-5p. The expression level of USP24 was inversely correlated with miR-139-5p expression in SSc patients and induced by TLR9 stimulation in healthy pDCs. These findings demonstrated that the miRNA profile is altered in pDCs of SSc patients already at early stages of the disease and indicate their potential contribution to pDC activation observed in patients. Full article
(This article belongs to the Special Issue Pathogenesis, Clinical Diagnosis, Treatments and Managements of Systemic Sclerosis)
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14 pages, 1885 KiB  
Article
Neutrophil Extracellular Traps Generation Relates with Early Stage and Vascular Complications in Systemic Sclerosis
by Kevin Didier, Delphine Giusti, Sebastien Le Jan, Christine Terryn, Celine Muller, Bach Nga Pham, Richard Le Naour, Frank D. Antonicelli and Amelie Servettaz
J. Clin. Med. 2020, 9(7), 2136; https://doi.org/10.3390/jcm9072136 - 7 Jul 2020
Cited by 32 | Viewed by 2820
Abstract
Systemic sclerosis (SSc) is a systemic disease characterized by a great clinical and immunological heterogeneity whose pathophysiology is still being unraveled. Recently, innate immunity has been proposed to participate to the pathogenesis of SSc. In this study, we investigated the release of neutrophil [...] Read more.
Systemic sclerosis (SSc) is a systemic disease characterized by a great clinical and immunological heterogeneity whose pathophysiology is still being unraveled. Recently, innate immunity has been proposed to participate to the pathogenesis of SSc. In this study, we investigated the release of neutrophil extracellular traps (NETs) according to patient phenotype. Polymorphonuclear neutrophils (PMN) from 34 SSc patients and 26 healthy controls were stimulated by serum from SSc or healthy subject. NETs were visualized using epifluorescence microscope after DNA, myeloperoxidase, and Histone H3 tagging. Area of NETs were quantified using an original macro running in ImageJ® software. PMN from SSc patients were significantly more prone to releasing NETs than control PMN after autologous stimulation. PMN from patients with severe vascular complications (pulmonary arterial hypertension, digital ulcers) produced more NETs than PMN from other SSc patients and their aberrant NET production appeared to be sustained over time. In patients with pulmonary interstitial disease or extensive cutaneous fibrosis, NET production was high at an early stage of the disease before progressively decreasing. Both serum factors and PMN activation status were involved in the enhanced production of NETs in SSc. Consequently, neutrophils and especially NETosis represent new physiopathological and therapeutic fields in SSc. Full article
(This article belongs to the Special Issue Pathogenesis, Clinical Diagnosis, Treatments and Managements of Systemic Sclerosis)
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Review

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25 pages, 439 KiB  
Review
Potential Biomarkers in Systemic Sclerosis: A Literature Review and Update
by Akira Utsunomiya, Noritaka Oyama and Minoru Hasegawa
J. Clin. Med. 2020, 9(11), 3388; https://doi.org/10.3390/jcm9113388 - 22 Oct 2020
Cited by 27 | Viewed by 4133
Abstract
Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by dysregulation of the immune system, vascular damage, and fibrosis of the skin and internal organs. Patients with SSc show a heterogeneous phenotype and a range of clinical courses. Therefore, biomarkers that are helpful [...] Read more.
Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by dysregulation of the immune system, vascular damage, and fibrosis of the skin and internal organs. Patients with SSc show a heterogeneous phenotype and a range of clinical courses. Therefore, biomarkers that are helpful for precise diagnosis, prediction of clinical course, and evaluation of the therapeutic responsiveness of disease are required in clinical practice. SSc-specific autoantibodies are currently used for diagnosis and prediction of clinical features, as other biomarkers have not yet been fully vetted. Krebs von den Lungen-6 (KL-6), surfactant protein-D (SP-D), and CCL18 have been considered as serum biomarkers of SSc-related interstitial lung disease. Moreover, levels of circulating brain natriuretic peptide (BNP) and N-terminal pro-brain natriuretic peptide (NT-proBNP) can provide diagnostic information and indicate the severity of pulmonary arterial hypertension. Assessment of several serum/plasma cytokines, chemokines, growth factors, adhesion molecules, and other molecules may also reflect the activity or progression of fibrosis and vascular involvement in affected organs. Recently, microRNAs have also been implicated as possible circulating indicators of SSc. In this review, we focus on several potential SSc biomarkers and discuss their clinical utility. Full article
(This article belongs to the Special Issue Pathogenesis, Clinical Diagnosis, Treatments and Managements of Systemic Sclerosis)
27 pages, 2644 KiB  
Review
The Pathogenesis of Systemic Sclerosis: An Understanding Based on a Common Pathologic Cascade across Multiple Organs and Additional Organ-Specific Pathologies
by Yoshihide Asano
J. Clin. Med. 2020, 9(9), 2687; https://doi.org/10.3390/jcm9092687 - 19 Aug 2020
Cited by 64 | Viewed by 12031
Abstract
Systemic sclerosis (SSc) is a multisystem autoimmune and vascular disease resulting in fibrosis of various organs with unknown etiology. Accumulating evidence suggests that a common pathologic cascade across multiple organs and additional organ-specific pathologies underpin SSc development. The common pathologic cascade starts with [...] Read more.
Systemic sclerosis (SSc) is a multisystem autoimmune and vascular disease resulting in fibrosis of various organs with unknown etiology. Accumulating evidence suggests that a common pathologic cascade across multiple organs and additional organ-specific pathologies underpin SSc development. The common pathologic cascade starts with vascular injury due to autoimmune attacks and unknown environmental factors. After that, dysregulated angiogenesis and defective vasculogenesis promote vascular structural abnormalities, such as capillary loss and arteriolar stenosis, while aberrantly activated endothelial cells facilitate the infiltration of circulating immune cells into perivascular areas of various organs. Arteriolar stenosis directly causes pulmonary arterial hypertension, scleroderma renal crisis and digital ulcers. Chronic inflammation persistently activates interstitial fibroblasts, leading to the irreversible fibrosis of multiple organs. The common pathologic cascade interacts with a variety of modifying factors in each organ, such as keratinocytes and adipocytes in the skin, esophageal stratified squamous epithelia and myenteric nerve system in gastrointestinal tract, vasospasm of arterioles in the heart and kidney, and microaspiration of gastric content in the lung. To better understand SSc pathogenesis and develop new disease-modifying therapies, it is quite important to understand the complex pathogenesis of SSc from the two distinct perspectives, namely the common pathologic cascade and additional organ-specific pathologies. Full article
(This article belongs to the Special Issue Pathogenesis, Clinical Diagnosis, Treatments and Managements of Systemic Sclerosis)
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