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Sclerosis
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Recent Advances in Understanding Systemic Sclerosis
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Recent Advances in Understanding Systemic Sclerosis

A special issue of Sclerosis (ISSN 2813-3064).

Deadline for manuscript submissions: 10 March 2025 | Viewed by 4623

Special Issue Editor

Prof. Dr. Jörg Christoph Henes

E-Mail Website
Guest Editor
Centre for Interdisciplinary Clinical Immunology, University of Tübingen, 72076 Tübingen, Germany
Interests: connective tissue diseases; systemic sclerosis; lupus; myositis; pregnancy, fertility and sexuality in autoimmune diseases; stem cell transplantation/CAR-T cell therapy in autoimmune diseases; familial Mediterranean fever

Special Issue Information

Dear Colleagues,

Systemic sclerosis (SSc) is a complex, multifaceted autoimmune disease characterized by vascular abnormalities, immune system dysregulation, and progressive fibrosis of the skin and internal organs. Despite significant strides in understanding the pathogenesis of SSc, it remains a challenging condition to diagnose and manage due to its heterogeneity and its variability in clinical manifestations.

The Special Issue “Recent Advances in Understanding Systemic Sclerosis” aims to highlight the latest research findings and clinical advancements in the field of SSc. Our goal is to provide a comprehensive overview of the current state of knowledge and to explore innovative therapeutic approaches that could improve patient outcomes.

Topics within the scope of this Special Issue include, but are not limited to, the following:

  • Diagnosis and assessment of SSc;
  • Pathophysiology of SSc;
  • The role of genetic and environmental factors in disease development;
  • Cutting-edge insights into the molecular mechanisms underlying fibrosis;
  • Novel and emerging therapies for SSc.

By bringing together leading experts and researchers, this Special Issue seeks to foster collaboration and stimulate further investigation into systemic sclerosis. We hope that the insights presented here will pave the way for improved diagnostic tools, more effective treatments, and ultimately, better quality of life for patients affected by this debilitating disease.

Dr. Jörg Christoph Henes
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Sclerosis is an international peer-reviewed open access quarterly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 1000 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • systemic sclerosis
  • systemic scleroderma
  • diagnosis
  • therapies
  • pathophysiology

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  • Greater discoverability: Special Issues support the reach and impact of scientific research. Articles in Special Issues are more discoverable and cited more frequently.
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Further information on MDPI's Special Issue polices can be found here.

Published Papers (4 papers)

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Review

20 pages, 1617 KiB  
Review
Preclinical and Clinical Data on Current Therapeutic Options for Micro- and Macrovascular Abnormalities in Systemic Sclerosis
by Konstantina Bakopoulou, Issa El Kaouri, Elina Siliogka, Periklis Siliogkas, Russka Shumnalieva and Tsvetelina Velikova
Sclerosis 2024, 2(4), 322-340; https://doi.org/10.3390/sclerosis2040021 - 29 Oct 2024
Viewed by 888
Abstract
Background: Systemic sclerosis (SSc) represents a multidimensional disease affecting various organs and systems, with the common denominator being the vascular pathology encountered in the micro- and macrocirculation of SSc patients. Recently, much progress has been made toward understanding the molecular basis of endothelial [...] Read more.
Background: Systemic sclerosis (SSc) represents a multidimensional disease affecting various organs and systems, with the common denominator being the vascular pathology encountered in the micro- and macrocirculation of SSc patients. Recently, much progress has been made toward understanding the molecular basis of endothelial injury and subsequent fibroblast activation, thus paving the way for specific therapy that can target and counteract these processes. Aim: In this review, we examined the latest preclinical and clinical data on therapeutic options to address vascular abnormalities in SSc. Results: We discuss the efficacy of current treatments, including pharmacological agents and emerging therapies, in mitigating vascular damage and improving patient outcomes based on preclinical models and clinical trials that offer evidence of their safety and effectiveness. Conclusions: Although promising therapeutic strategies emerge, optimizing the management of vascular abnormalities in SSc requires further research. Full article
(This article belongs to the Special Issue Recent Advances in Understanding Systemic Sclerosis)
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12 pages, 2053 KiB  
Review
Insights and Future Perspectives in Calcinosis Cutis Associated with Systemic Sclerosis
by Luna Lazar, Mette Mogensen, Mikael Ploug Boesen and Anne Braae Olesen
Sclerosis 2024, 2(4), 302-313; https://doi.org/10.3390/sclerosis2040019 - 10 Oct 2024
Viewed by 811
Abstract
Introduction: Calcinosis cutis (CC), the pathological deposition of calcium salts in the skin, is a frequent and challenging complication of systemic sclerosis (SSc). Despite its high prevalence, the underlying pathophysiology remains poorly understood, complicating treatment strategies. Material and Methods: This narrative review synthesizes [...] Read more.
Introduction: Calcinosis cutis (CC), the pathological deposition of calcium salts in the skin, is a frequent and challenging complication of systemic sclerosis (SSc). Despite its high prevalence, the underlying pathophysiology remains poorly understood, complicating treatment strategies. Material and Methods: This narrative review synthesizes the literature on CC in the context of SSc. The current understanding and treatment of CC in SSc is reviewed, focusing on the role of hypoxia in its pathogenesis and the therapeutic potential of sodium thiosulfate (STS). Results and Discussion: Research indicates a potential link between hypoxia and the development of CC in SSc, shedding light on novel pathogenic mechanisms. Additionally, promising results from treatments such as STS spurs interest in conducting larger, randomized controlled trials to validate these findings. Full article
(This article belongs to the Special Issue Recent Advances in Understanding Systemic Sclerosis)
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14 pages, 655 KiB  
Review
A Narrative Review of Therapeutic Options in Systemic Sclerosis Associated Interstitial Lung Disease
by Robert Harrington, Patricia Harkins and Richard Conway
Sclerosis 2024, 2(4), 288-301; https://doi.org/10.3390/sclerosis2040018 - 30 Sep 2024
Viewed by 722
Abstract
Background: Interstitial lung disease (ILD) has replaced scleroderma renal crisis as the leading cause of mortality in systemic sclerosis (SSc), with a 10-year mortality of 40%. There have been well-powered randomised control trials (RCTs) demonstrating the effect of cyclophosphamide (CYC), mycophenolic acid (MMF), [...] Read more.
Background: Interstitial lung disease (ILD) has replaced scleroderma renal crisis as the leading cause of mortality in systemic sclerosis (SSc), with a 10-year mortality of 40%. There have been well-powered randomised control trials (RCTs) demonstrating the effect of cyclophosphamide (CYC), mycophenolic acid (MMF), nintedanib and tocilizumab (TCZ) in SSc-ILD but a paucity of sufficiently powered studies investigating other agents in the disease. Methods: This is a narrative review which examines the existing evidence for immunosuppressive treatments, transplant and adjunctive therapies in SSc-ILD by reviewing the key landmark trials in the last two decades. Results: MMF for 2 years is as effective as oral CYC for 1 year. Rituximab (RTX) is non-inferior to CYC. TCZ appears to have a beneficial effective regardless of the extent of lung involvement. Conclusions: There is now a strong evidence base supporting the use of MMF as the first line option in SSc-ILD. RTX, CYC and TCZ are viable therapeutic options if there is ILD progression on MMF. Anti-fibrotic and pulmonary arterial (PAH) treatments likely add long-term synergistic benefits. There remains a role for lung transplantation in select patients. Full article
(This article belongs to the Special Issue Recent Advances in Understanding Systemic Sclerosis)
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18 pages, 4823 KiB  
Review
Lung Involvement in Systemic Sclerosis—From Pathogenesis to Prediction
by Issa El Kaouri, Konstantina Bakopoulou, Ivan Padjen, Velik Lazarov, Paraskevas Panagiotis Sdralis, Tsvetelina Velikova and Russka Shumnalieva
Sclerosis 2024, 2(3), 199-216; https://doi.org/10.3390/sclerosis2030014 - 17 Aug 2024
Viewed by 1115
Abstract
Systemic sclerosis (SSc) is a rare, multifactorial autoimmune disease characterized by widespread vascular damage and fibrosis. Pulmonary involvement is a significant manifestation of SSc, contributing to considerable morbidity and mortality. Therefore, identifying reliable biomarkers is of the utmost importance. This review explores emerging [...] Read more.
Systemic sclerosis (SSc) is a rare, multifactorial autoimmune disease characterized by widespread vascular damage and fibrosis. Pulmonary involvement is a significant manifestation of SSc, contributing to considerable morbidity and mortality. Therefore, identifying reliable biomarkers is of the utmost importance. This review explores emerging biomarkers to enhance diagnostic accuracy, prognostic assessment, and disease monitoring in SSc lung involvement. We discuss recent findings in immunological biomarkers, inflammatory indicators, and other parameters that can function as potential diagnostic and prognostic tools. A comprehensive understanding of these biomarkers could result in earlier and more accurate detection of pulmonary complications in SSc, aiding in timely intervention. Furthermore, we explore the advances in disease monitoring through innovative biomarkers, focusing on their roles in disease activity and treatment response. Integrating these novel biomarkers into current clinical practice and therapeutic protocols through clinical trials can revolutionize the management of SSc-related lung disease, ultimately improving patient outcomes and quality of life. Full article
(This article belongs to the Special Issue Recent Advances in Understanding Systemic Sclerosis)
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