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News in the Pathogenesis, Diagnosis and Treatment of Systemic Sclerosis

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

Deadline for manuscript submissions: 20 April 2026 | Viewed by 3616

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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

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Keywords

systemic sclerosis
scleroderma
capillaroscopy
autoantibodies
pathogenesis

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Published Papers (4 papers)

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Research

14 pages, 265 KB

Open AccessArticle

Association of Inflammation-Based Ratios with Endothelial Dysfunction Markers and Clinical Parameters in Limited Cutaneous Systemic Sclerosis

by Leyla Schweiger, Andreas Meinitzer, Heimo Strohmaier, Florentine Moazedi-Fürst, Viktoria Nemecz, Katharina Kurzmann-Gütl, Marianne Brodmann, Franz Hafner and Philipp Jud

J. Clin. Med. 2025, 14(24), 8806; https://doi.org/10.3390/jcm14248806 - 12 Dec 2025

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Abstract

Background: Limited cutaneous systemic sclerosis (lcSSc) is an autoimmune disease with a wide range of different biomarkers, while inflammation-based ratios have been less extensively investigated. This study aimed to evaluate the associations between inflammation-based ratios, disease-specific parameters, and endothelial dysfunction, as well as to assess the predictive role of inflammation-based ratios in lcSSc. Methods: A total of 38 lcSSc patients and 38 matched controls with primary Raynaud’s phenomenon were analyzed at baseline regarding inflammation-based ratios, lcSSc-specific parameters, and parameters of endothelial dysfunction. LcSSc patients were prospectively observed during a 3-year follow-up period in which lcSSc complications were recorded annually. Results: LcSSc patients had a significantly higher neutrophil-to-lymphocyte ratio, monocyte-to-lymphocyte ratio (MLR), fibrinogen-to-albumin ratio, monocyte/high-density lipoprotein (HDL) ratio, and neutrophil/HDL ratio versus controls (all p < 0.05). During follow-up, the MLR, C-reactive protein (CRP)/albumin ratio, monocyte/HDL ratio, and neutrophil/HDL ratio increased significantly (all p < 0.05) in lcSSc patients. The monocyte/HDL ratio correlated positively with the DETECT score step 2 (r = 0.453, p = 0.032) and negatively with the UCLA SCTC GIT total score (r = −0.469, p = 0.024). The CRP/albumin ratio correlated significantly with the EUSTAR index (r = 0.473, p = 0.024) and the fibrinogen-to-albumin ratio correlated with asymmetric dimethylarginine (r = 0.452, p = 0.044). The MLR and CRP/albumin ratio were associated with development of pulmonary arterial hypertension (p = 0.036, p = 0.006), and the lymphocyte/HDL ratio was associated with newly developed interstitial lung disease (p = 0.004). Conclusions: Readily available inflammation-based ratios may reflect vascular and inflammatory activity and could contribute to risk stratification for pulmonary complications in lcSSc; however, these exploratory findings require confirmation in larger cohorts. Full article

(This article belongs to the Special Issue News in the Pathogenesis, Diagnosis and Treatment of Systemic Sclerosis)

14 pages, 363 KB

Open AccessArticle

Change in Antinuclear Antibodies After Lung Transplantation in Patients with Systemic Sclerosis

by Víctor Barreales-Rodríguez, Alfredo Guillen-Del-Castillo, Cristina Berastegui, Manuel López-Meseguer, Víctor Monforte, Berta Saez-Gimenez, Ana Villar, Iñigo Ojanguren, Claudia Codina-Clavaguera, Alejandra Fernández-Luque, María Teresa Sanz-Martínez, Laura Viñas-Giménez, Janire Perurena-Prieto, Laura Triginer-Gil, Luis Alcalá-González, Carlos Bravo and Carmen Pilar Simeón Aznar

J. Clin. Med. 2025, 14(24), 8673; https://doi.org/10.3390/jcm14248673 - 7 Dec 2025

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Abstract

Objectives: Lung transplantation (LT) is a rescue therapy for end-stage pulmonary diseases, including systemic autoimmune diseases. The aim of this study was to analyse the evolution of patients with systemic sclerosis (SSc) who, after undergoing LT, become negative for antinuclear antibodies (ANA) and to assess whether they have different clinical and prognostic characteristics than patients who do not become negative. Material and Methods: A retrospective, descriptive analysis was performed over a cohort of patients with a diagnosis of SSc, who underwent unilateral or bilateral LT between 2006 and 2021 at the Vall d’Hebron University Hospital. Clinical and analytical data were obtained from these patients by reviewing their electronic medical records. Two groups of patients were compared: those who tested negative for ANA after LT and those who did not. Statistical analysis was performed with SPSS Statistics 20.0. Results: Eighteen patients were included. The most frequent indication for LT was interstitial lung disease (ILD) combined with pulmonary hypertension (PH), in 13 (72%) patients. All had ANA before the LT (n = 18), and regarding specific SSc autoantibodies, anti-topoisomerase I was presented in 44% (n = 8), anti-U11/U12RNP in 17% (n = 3), anti-RNA Polymerase III in 11.1% (n = 2), anti-Ro52 in 11% (n = 2) and anti-centromere in 6% of individuals (n = 1). 39% (n = 7) of the patients had negative post-LT ANA, 44% (n = 8) had declining titres, and 17% (n = 3) had stable ANA titres. Titres did not increase in any case after LT. Those patients who became ANA-negative after LT were those who had significantly lower titres before LT. No statistically significant differences between groups were found related to pre-LT clinical characteristics, immunosuppressive regimen applied after LT, or in post-LT outcomes. A non-significant trend towards better survival was observed in patients who became ANA negative, with a cumulative survival at 5 years of 85.7% compared to 72.7% among those who remained ANA-positive. Conclusions: Most patients with SSc clear ANA or reduce their levels after LT. A trend towards better survival was observed in this group, compared to the group of transplanted patients who remained positive. Full article

(This article belongs to the Special Issue News in the Pathogenesis, Diagnosis and Treatment of Systemic Sclerosis)

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14 pages, 2207 KB

Open AccessArticle

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

Cited by 3 | Viewed by 1530

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 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 KB

Open AccessFeature PaperArticle

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 2 | Viewed by 1048

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 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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