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News in the Pathogenesis, Diagnosis and Treatment of Systemic Sclerosis
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News in the Pathogenesis, Diagnosis and Treatment of Systemic Sclerosis

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

Deadline for manuscript submissions: 24 October 2025 | Viewed by 1637

Special Issue Editors

Dr. Mayka Freire

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Guest Editor
Systemic Autoimmune Diseases Unit, Internal Medicine Department, Hospital Clínico de Santiago de Compostela, 15706 Santiago de Compostela, Spain
Interests: capillaroscopy; systemic sclerosis; systemic autoimmune diseases; clinical immunology; connective tissue diseases
Dr. Bernardo Sopeña

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Guest Editor
Departamento de Psiquiatría, Radiología, Salud Pública, Enfermería y Medicina, Facultad de Medicina, Universidad de Santiago de Compostela, 15705 Santiago de Compostela, Spain
Interests: systemic autoimmune diseases; clinical Immunology; connective tissue diseases; vasculitis

Special Issue Information

Dear Colleagues,

Systemic sclerosis (SS) is a systemic autoimmune disease whose origin is unknown, although everything points to a multifactorial origin in which both genetic and environmental factors are involved. Its diagnosis can also be quite a challenge, given its great variability in phenotypic expression. Currently, SS has no cure, although there are treatments to alleviate the conditions that may appear in the different organs. In recent years, mortality from scleroderma renal crisis or cardiopulmonary involvement, mainly due to pulmonary hypertension or interstitial lung disease, has improved substantially in these patients, due to the development of specific treatments. However, even today, it is the systemic autoimmune disease with the worst prognosis, since it drastically reduces the quality of life and the life expectancy of patients who suffer from it is 16–34 years less than the general population. For all these reasons, SS has a great social and psychological impact on those affected and their families.

Research into this entity, although it has advanced in recent years, is clearly insufficient to respond to the many doubts that still exist regarding its pathogenesis, diagnosis and treatment.

We are pleased to invite you to contribute research to this Special Issue that we are preparing on SS. Original research articles and reviews are welcome. Research areas may include (but not limited to) the following: environmental influence on the pathogenesis of ES; developments in periungual capillaroscopy; visceral involvement and its correlation with different antibodies; new antibodies related to the disease; new therapeutic pathways.

We look forward to receiving your contributions.

Dr. Mayka Freire
Dr. Bernardo Sopeña
Guest Editors

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. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly 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 2600 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
  • scleroderma
  • capillaroscopy
  • autoantibodies
  • pathogenesis

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Published Papers (2 papers)

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Research

14 pages, 2207 KiB  
Article
Serum Proteomic Markers in Patients with Systemic Sclerosis in Relation to Silica Exposure
by Mayka Freire, Bernardo Sopeña, Susana Bravo, Carlos Spuch, Ana Argibay, Melania Estévez, Carmen Pena, Martín Naya, Adela Lama and Arturo González-Quintela
J. Clin. Med. 2025, 14(6), 2019; https://doi.org/10.3390/jcm14062019 - 16 Mar 2025
Viewed by 753
Abstract
Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease characterised by fibrosis, vasculopathy, and immune dysfunction. Silica exposure has been associated with a more aggressive phenotype of the disease, including diffuse cutaneous involvement and interstitial lung disease. This study aims to identify proteomic [...] Read more.
Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease characterised by fibrosis, vasculopathy, and immune dysfunction. Silica exposure has been associated with a more aggressive phenotype of the disease, including diffuse cutaneous involvement and interstitial lung disease. This study aims to identify proteomic differences between SSc patients exposed to silica and those not exposed to silica. Methods: An observational study of 32 SSc patients (11 silica-exposed and 21 non-exposed) was performed, with occupational history and quantitative proteomic analysis using SWATH-MS mass spectrometry. Differentially expressed proteins were analysed, and functional pathway enrichment was performed. Results: Eight proteins showed significant differences between groups, all with reduced levels in silica-exposed patients: adiponectin, immunoglobulins (IGLV3-19, IGLV2-18), complement C2, alpha-2-macroglobulin, vitronectin, cytoplasmic actin 2, and pigment epithelium-derived factor. Alterations in pathways related to fibrinolysis, complement activation, and inflammation were highlighted, suggesting that silica exposure may influence the pathogenesis of SSc and worsen its clinical course. Conclusions: This study supports the hypothesis that silica exposure is not only a triggering factor for SSc, but is also modulating its progression through inflammatory, procoagulant, and fibrotic pathways. The identification of proteomic biomarkers could contribute to the phenotypic classification of patients and the development of personalised therapies. Future studies should expand the cohort and further investigate the functional mechanisms of these proteins in SSc. Full article
(This article belongs to the Special Issue News in the Pathogenesis, Diagnosis and Treatment of Systemic Sclerosis)
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13 pages, 259 KiB  
Article
Associations Between Soluble Cell Adhesion Molecules and Cardiovascular Comorbidities in Systemic Sclerosis: Implications for Insulin Resistance
by Iván Ferraz-Amaro, Zeina Ibrahim-Achi, Antonia de Vera-González, Alejandra González-Delgado, Mónica Renuncio-García, Esther F. Vicente-Rabaneda, J. Gonzalo Ocejo-Vinyals, Santos Castañeda and Miguel Á. González-Gay
J. Clin. Med. 2025, 14(5), 1467; https://doi.org/10.3390/jcm14051467 - 22 Feb 2025
Cited by 1 | Viewed by 523
Abstract
Background: Soluble cell adhesion molecules such as sICAM-1 (soluble intercellular adhesion molecule-1), sVCAM-1 (soluble vascular cell adhesion molecule-1), and P-selectin have been implicated in cardiovascular disease pathogenesis in the general population. Cardiovascular disease is prevalent among patients with systemic sclerosis (SSc). This [...] Read more.
Background: Soluble cell adhesion molecules such as sICAM-1 (soluble intercellular adhesion molecule-1), sVCAM-1 (soluble vascular cell adhesion molecule-1), and P-selectin have been implicated in cardiovascular disease pathogenesis in the general population. Cardiovascular disease is prevalent among patients with systemic sclerosis (SSc). This study aims to investigate potential associations between the serum levels of these adhesion molecules and specific cardiovascular comorbidities in SSc patients. Methods: This cross-sectional study encompassed 81 individuals with SSc. All SSc patients underwent a complete clinical evaluation. Serum sICAM-1, sVCAM-1, and P-selectin levels, lipid profiles and insulin resistance indices, and carotid ultrasound were assessed. Multivariable linear regression analyses were employed to investigate potential associations between adhesion molecule levels (sICAM, sVCAM, and P-selectin) and both SSc-specific manifestations and cardiometabolic parameters. Results: The associations of disease-related parameters with sICAM-1, sVCAM-1, and P-selectin levels were limited. Notably, only the modified Rodnan skin score exhibited a significant positive association with sVCAM-1 levels, while no such associations were observed for sICAM-1 and P-selectin. Regarding cardiovascular disease-related data, sVCAM-1 significantly correlated with higher values of insulin resistance and beta-cell function indices. In the case of P-selectin, although a trend was observed, statistical significance was not reached. Conclusions: In patients with SSc, serum values of sVCAM-1 independently correlate with insulin resistance. The assessment of CAMs in patients with SSc could serve as a valuable clinical tool for identifying individuals with increased insulin resistance and a higher risk of cardiovascular disease. Full article
(This article belongs to the Special Issue News in the Pathogenesis, Diagnosis and Treatment of Systemic Sclerosis)
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