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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: 30 May 2025 | Viewed by 10366

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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  • Expansion of research network: Special Issues facilitate connections among authors, fostering scientific collaborations.
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Further information on MDPI's Special Issue policies can be found here.

Published Papers (7 papers)

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Research

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16 pages, 510 KiB  
Article
Uncovering Subclinical Cardiac Involvement in VEDOSS: An Echocardiographic Driven Study
by Eugenio Capparelli, Eleonora Zaccara, Ilaria Suardi, Antonella Laria, Laura Castelnovo, Eleonora Mauric, Daniela Bompane, Antonio Tamburello, Maria Iacovantuono, Maria Sole Chimenti, Antonino Mazzone and Paola Faggioli
Sclerosis 2025, 3(1), 7; https://doi.org/10.3390/sclerosis3010007 - 25 Feb 2025
Viewed by 323
Abstract
Background: The 2011 Very Early Diagnosis of Systemic Sclerosis (VEDOSS) criteria include both patients at risk of progression and those with mild non-progressive forms of SSc. Early diastolic and systolic dysfunction can indicate myocardial fibrosis in SSc patients, yet data on myocardial impairment [...] Read more.
Background: The 2011 Very Early Diagnosis of Systemic Sclerosis (VEDOSS) criteria include both patients at risk of progression and those with mild non-progressive forms of SSc. Early diastolic and systolic dysfunction can indicate myocardial fibrosis in SSc patients, yet data on myocardial impairment in the VEDOSS population are limited. Objectives: This study aimed to identify subclinical echocardiographic changes and predictive markers of cardiac dysfunction in both very early and mild-longstanding forms of VEDOSS. Methods: We conducted a cross-sectional observational study involving 61 patients meeting VEDOSS criteria followed up regularly within our Scleroderma referral center. Patients were categorized as early VEDOSS (e-VEDOSS) or mild-longstanding VEDOSS (ml-VEDOSS) based on disease duration (≥10 years). We analyzed clinical and demographic data, focusing on echocardiographic parameters such as the E/A ratio and left ventricular (LV) thickness. Statistical analyses included chi-square, Fischer exact, and student’s t tests, with a significance threshold of p < 0.05. Results: ml-VEDOSS patients were older and reported a higher burden of comorbidities. Autoantibody-positive patients exhibited lower E/A ratios and increased left atrial size. Late nailfold videocapillaroscopic pattern patients exhibited increased PWED thickening and aortic valve insufficiency. Notably, patients undergoing vasodilators experienced larger right atrial volume, while patients receiving Renin-Angiotensin-Aldosterone System (RAAS) inhibitors reported reduced E/A ratio. Multivariable analysis confirmed DLCO% as the sole predictor of both diastolic and systolic impairment in VEDOSS population. Conclusions: Careful monitoring of cardiac function in VEDOSS patients is crucial as subclinical alterations may occur even in the absence of symptoms. DLCO% emerged as an important predictor of LV diastolic dysfunction. Full article
(This article belongs to the Special Issue Recent Advances in Understanding Systemic Sclerosis)
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Review

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18 pages, 317 KiB  
Review
Biomarkers in Systemic Sclerosis
by Claudio Karsulovic and Lia Hojman
Sclerosis 2025, 3(2), 11; https://doi.org/10.3390/sclerosis3020011 - 30 Mar 2025
Viewed by 223
Abstract
Systemic sclerosis (SSc) is a complex autoimmune disease characterized by vasculopathy, immune dysregulation, and progressive fibrosis affecting the skin and internal organs. Pulmonary complications, including interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH), are major contributors to morbidity and mortality, while skin [...] Read more.
Systemic sclerosis (SSc) is a complex autoimmune disease characterized by vasculopathy, immune dysregulation, and progressive fibrosis affecting the skin and internal organs. Pulmonary complications, including interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH), are major contributors to morbidity and mortality, while skin fibrosis remains a hallmark of disease heterogeneity. Despite advances in understanding SSc pathogenesis, early diagnosis and timely therapeutic intervention remain challenging due to the rapid progression of inflammation and the narrow window before irreversible fibrosis occurs. The identification of reliable biomarkers is crucial for improving diagnosis, monitoring disease activity, and guiding treatment decisions in SSc. While autoantibodies are well-established diagnostic tools, this review focused on non-autoantibody biomarkers, including soluble proteins, cytokines, chemokines, epigenetic modifiers, and oxidative stress indicators. These biomarkers reflect diverse pathogenic mechanisms such as endothelial injury, fibroblast activation, immune signaling, and extracellular matrix remodeling. By examining the available evidence across both clinical and preclinical studies, this review provides an updated overview of molecular markers involved in inflammation and fibrosis in SSc. Understanding their biological significance and therapeutic potential may improve risk stratification, guide targeted interventions, and ultimately contribute to the development of precision medicine strategies in systemic sclerosis. Full article
(This article belongs to the Special Issue Recent Advances in Understanding Systemic Sclerosis)
10 pages, 215 KiB  
Review
B-Cell-Depleting Immune Therapies as Potential New Treatment Options for Systemic Sclerosis
by Gerhard Zugmaier, Matthias Klinger, Marion Subklewe, Faraz Zaman and Franco Locatelli
Sclerosis 2025, 3(1), 5; https://doi.org/10.3390/sclerosis3010005 - 26 Jan 2025
Cited by 1 | Viewed by 1215
Abstract
Background: Systemic sclerosis (SSc), also known as scleroderma, is a complex, chronic autoimmune disease characterized by fibrosis of the skin and internal organs, vasculopathy, and immune system dysregulation. The treatment of SSc has historically focused on symptom management and slowing down disease progression [...] Read more.
Background: Systemic sclerosis (SSc), also known as scleroderma, is a complex, chronic autoimmune disease characterized by fibrosis of the skin and internal organs, vasculopathy, and immune system dysregulation. The treatment of SSc has historically focused on symptom management and slowing down disease progression through conventional immune-suppressive agents. New therapeutic approaches have been emerging due to advances in understanding of the disease mechanisms, particularly in the areas of fibrosis, vascular involvement, and immune dysregulation. Methods: In this review of the literature, we discuss the current stage of development of B-cell-depleting immune therapies in SSc. Results: B-cell depletion therapy has become an area of growing interest in the treatment of SSc due to the role played by B cells in the pathogenesis of the disease. There is increasing evidence that B cells contribute to disease progression through multiple mechanisms. B cells in SSc are implicated in autoantibody production, cytokine production, and fibroblast activation. B cells are responsible for producing autoantibodies, such as anti-topoisomerase I (Scl-70) and anti-centromere antibodies, which are hallmarks of SSc. B cells release pro-inflammatory cytokines (such as interleukin-6 [IL-6] and transforming growth factor β [TGF-β]), which promote fibrosis and inflammation, they also contribute to the activation of fibroblasts, the cells responsible for excessive collagen production and fibrosis, a key feature of SSc. Conclusions: In light of these findings, therapies that target B cells are being investigated for their potential to modify the disease course in SSc, particularly by reducing autoantibody production, inflammation, and fibrosis. Full article
(This article belongs to the Special Issue Recent Advances in Understanding Systemic Sclerosis)
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 1517
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 1751
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 1485
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 2079
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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