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Systemic Sclerosis: New Insight into Pathogenesis, Molecular and Immune Mechanisms

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: closed (30 April 2025) | Viewed by 829

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Guest Editor
Department of Internal Medicine, Rheumatology and Clinical Immunology, Medical Faculty in Katowice, Medical University of Silesia, 40-055 Katowice, Poland
Interests: heart failure; rheumatoid arthritis; tumor necrosis factor; TNF inhibitors
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Special Issue Information

Dear Colleagues,

Systemic sclerosis, a connective tissue disease that lies at the intersection of autoimmunity and extensive fibrotic processes, is attracting increasing attention from researchers. Despite the significant progress achieved in our understanding of the mechanisms underlying autoimmunity, inflammation, and fibrosis, many pathophysiological aspects of this disease remain unresolved. This Special Issue will provide a platform for sharing the latest advances in the pathophysiology, treatment, and clinical manifestations of systemic sclerosis. Special emphasis will be placed on the molecules involved in profibrotic signaling, disease biomarkers, and genetic markers. We also welcome clinical studies providing up-to-date insights into patient characteristics, as well as the impact of comorbidities on the development and progression of the disease. Clinical and laboratory studies, as well as review papers, are encouraged.

Prof. Dr. Przemysław Kotyla
Guest Editor

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Keywords

  • systemic sclerosis
  • autoimmunity
  • inflammation
  • fibrosis
  • pathophysiology and treatment
  • profibrotic signaling
  • biomarkers

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Published Papers (2 papers)

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Research

16 pages, 640 KiB  
Article
The Potential of Cardiac Biomarkers in Differentiating Disease Subtypes in Patients with Systemic Sclerosis: Focus on GDF15, MR-pro ANP, and suPAR
by Olga Gumkowska-Sroka, Anna Chudek, Aleksander Owczarek, Kornelia Kuźnik-Trocha, Kacper Kotyla, Jan Kurdybacha, Jerzy Chudek, Katarzyna Komosińska-Vassev, Katarzyna Winsz-Szczotka, Krystyna Olczyk and Przemysław Kotyla
Int. J. Mol. Sci. 2025, 26(9), 3938; https://doi.org/10.3390/ijms26093938 - 22 Apr 2025
Viewed by 134
Abstract
Systemic sclerosis, a connective tissue disease of unknown etiology and unpredictable outcomes, is characterized by the fibrosis of the skin and internal organs, vasculopathy, and immune system dysregulation. The disease is classified into two main subtypes, which differ in clinical presentation, complications, and [...] Read more.
Systemic sclerosis, a connective tissue disease of unknown etiology and unpredictable outcomes, is characterized by the fibrosis of the skin and internal organs, vasculopathy, and immune system dysregulation. The disease is classified into two main subtypes, which differ in clinical presentation, complications, and prognosis. While several biomarkers have been proposed to distinguish between these subtypes, none have achieved high sensitivity and specificity. The search for dependable markers that can differentiate between the two primary subtypes of systemic sclerosis continues. To address this gap, our study evaluated the utility of novel cardiac biomarkers, including growth differentiation factor 15 (GDF15), galectin-3, mid-regional pro-atrial natriuretic peptide (MR-proANP), glutathione S-transferase π, mid-regional adrenomedullin, and soluble urokinase plasminogen activator receptor (suPAR), in a cohort of 79 patients with both lcSSc and dSSc subtypes. The results demonstrated a significant elevation of GDF15 (medians: 2.07 vs. 1.10 ng/L; p < 0.001) and MR-proANP (92.55 vs. 65.60 pmol/L; p < 0.05) levels in SSc patients compared to healthy controls. Moreover, GDF15 (1.65 vs. 2.34 ng/mL; p < 0.05), MR-proANP (80.87 vs. 109.27 pmol/L; p < 0.05), and suPAR (1.83 vs. 2.44 ng/mL; p < 0.05) levels were notably higher in patients with dSSc compared to those with lcSSc. In the ROC analysis, only GDF-15, MR-proANP, and suPAR proved to have a statistically significant area under the curve (AUC). Patients with the GDF-15 ≥ 2182 ng/mL, MR-prANP ≥ 85.808 pmol/L, and suPAR ≥ 2.315 ng/mL have more than six-, eight-, and seven-times-higher odds for dcSSc, respectively. These findings highlight the potential of GDF15, suPAR, and MR-proANP as biomarkers for differentiating between the two main subtypes of systemic sclerosis. Full article
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15 pages, 2418 KiB  
Article
Comparative Evaluation of Bleomycin- and Collagen-V-Induced Models of Systemic Sclerosis: Insights into Fibrosis and Autoimmunity for Translational Research
by Lőrinc Nagy, Gábor Nagy, Tamás Juhász, Csaba Fillér, Gabriella Szűcs, Zoltán Szekanecz, György Vereb, Péter Antal-Szalmás and Árpád Szöőr
Int. J. Mol. Sci. 2025, 26(6), 2618; https://doi.org/10.3390/ijms26062618 - 14 Mar 2025
Viewed by 476
Abstract
Systemic sclerosis (SSc) is a complex autoimmune disease characterized by fibrosis, immune dysregulation, and vascular dysfunction, yet its pathogenesis remains incompletely understood. This study compares two widely used animal models of SSc—the bleomycin-induced fibrosis model and the collagen-V-induced autoimmune model—to evaluate their ability [...] Read more.
Systemic sclerosis (SSc) is a complex autoimmune disease characterized by fibrosis, immune dysregulation, and vascular dysfunction, yet its pathogenesis remains incompletely understood. This study compares two widely used animal models of SSc—the bleomycin-induced fibrosis model and the collagen-V-induced autoimmune model—to evaluate their ability to replicate key disease features. In the bleomycin model, consistent cardiac fibrosis was observed across treatment groups despite variability in fibrosis in the skin and lungs, suggesting organ-specific differences in susceptibility. The collagen-V model demonstrated robust autoantibody production against collagen-V, confirming its utility in studying immune activation, though fibrosis was largely confined to the heart. While the bleomycin model excels at mimicking rapid fibrosis and is suitable for testing antifibrotic therapies, the collagen-V model provides insights into antigen-specific autoimmunity. Both models highlight the dynamic nature of fibrosis, where ECM deposition and degradation occur concurrently, complicating its use as a quantitative disease marker. Cardiac fibrosis emerged as a consistent feature in both models, emphasizing its relevance in SSc pathophysiology. Combining these models or refining their design through hybrid approaches, extended timelines, or sex and age adjustments could enhance their translational utility. These findings advance understanding of SSc mechanisms and inform therapeutic development for this challenging disease. Full article
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