Effect of UV-A1 Phototherapy Treatment on Scleroderma: A Systematic Review

Cureus, Journal Year: 2025, Volume and Issue: unknown

Published: April 24, 2025

Language: Английский

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The efficacy and safety of divozilimab in patient with systemic sclerosis: 48-week results of the randomized double-blind placebo-controlled phase III clinical study LIBERIUS

Rheumatology Science and Practice, Journal Year: 2025, Volume and Issue: 63(2), P. 158 - 167

Published: May 1, 2025

The aim of the study is to evaluate efficacy, safety and immunogenicity divozilimab (DIV), anti-CD20 monoclonal antibody, in patients with systemic sclerosis (SS). Materials methods . Patients SS according ACR/EULAR (American College Rheumatology/ European Alliance Associations for Rheumatology) 2013 criteria modified Rodnan skin score (mRSS) ≥10 ≲20 forced vital capacity (FVC) ≥40% from due took part study. Infusions DIV 250 mg were administered on weeks 0 2, 500 – week 24 subsequent use open mode, starting 48. This publication presents data obtained 48 trial (before infusion at 48). Primary endpoint was change mRSS baseline dynamic FVC estimated as secondary endpoint. evaluation included frequency profile adverse events reactions (ARs). Immunogenicity assessed by detection binding neutralizing anti-drug antibodies Results 151 randomized into two groups: ( n =76) Placebo =75). most female; median duration disease about 3–4 years. initial value 14 13 points PBO groups, respectively. –5.8±1.1 group –2.7±1.0 (adjusted mean difference (AMD) 95% confidence interval –3.1 (–4.5; –1.7); p <0.0001). lung function stable treated DIV. A comparable demonstrated. frequent ARs a decrease number lymphocytes. There no severe serious group. All mild moderate. 5.3% (4/76) had without activity. Conclusion Divosilimab has demonstrated significant severity fibrosis, positive effect respiratory favorable profile, which allows consider it promising therapeutic option SS.

Language: Английский

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An interleukin 6 responsive plasma cell signature is associated with disease progression in systemic sclerosis interstitial lung disease

iScience, Journal Year: 2023, Volume and Issue: 26(11), P. 108133 - 108133

Published: Oct. 5, 2023

Systemic sclerosis (SSc) interstitial lung disease (ILD) is among the leading causes of SSc-related morbidity and mortality. Tocilizumab (TCZ, anti-IL6RA) has demonstrated a reduced rate pulmonary function decline in two phase 2/3 trials (faSScinate focuSSced) SSc-ILD patients. We performed transcriptome analysis skin biopsy samples collected studies to decipher gene networks that were potentially associated with clinical responses TCZ treatment. One module correlated progression showed pharmacodynamic changes treatment, was characterized by plasma cell (PC) genes. PC signature expression levels also significantly increased both fibrotic SSc IPF lungs compared controls. scRNAseq analyses confirmed genes co-expressed CD38 CD138 expressing subsets lungs. These data provide insights into potential role mechanisms action diseases.

Language: Английский

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Autoantibodies Targeting G-Protein-Coupled Receptors: Pathogenetic, Clinical and Therapeutic Implications in Systemic Sclerosis

International Journal of Molecular Sciences, Journal Year: 2024, Volume and Issue: 25(4), P. 2299 - 2299

Published: Feb. 15, 2024

Systemic sclerosis (SSc) is a multifaceted connective tissue disease whose aetiology remains largely unknown. Autoimmunity thought to play pivotal role in the development of disease, but direct pathogenic SSc-specific autoantibodies be established. The recent discovery functional antibodies targeting G-protein-coupled receptors (GPCRs), presence has been demonstrated different autoimmune conditions, shed some light on SSc pathogenesis. These bind GPCRs expressed immune and non-immune cells as their endogenous ligands, exerting either stimulatory or inhibitory effect corresponding intracellular pathways. Growing evidence suggests that, SSc, anti-GPCRs correlates with specific clinical manifestations. Autoantibodies endothelin receptor type A (ETAR) angiotensin 1 (AT1R) are associated severe vasculopathic SSc-related manifestations, while anti-C-X-C motif chemokine (CXCR) seem predictive interstitial lung involvement; anti-muscarinic-3 acetylcholine (M3R) have found patients gastrointestinal involvement anti-protease-activated (PAR1) detected experiencing scleroderma renal crisis. This review aims clarify potential pathogenetic significance GPCR-targeting focusing associations manifestations scleroderma. An extensive examination autoimmunity might provide valuable insights into underlying mechanisms thus enabling novel therapeutic strategies tailored target GPCR-mediated

Language: Английский

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Differentially expressed genes in systemic sclerosis: Towards predictive medicine with new molecular tools for clinicians

Autoimmunity Reviews, Journal Year: 2023, Volume and Issue: 22(6), P. 103314 - 103314

Published: March 12, 2023

Language: Английский

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Identification of lncRNA–miRNA–mRNA networks in circulating exosomes as potential biomarkers for systemic sclerosis

Frontiers in Medicine, Journal Year: 2023, Volume and Issue: 10

Published: Feb. 16, 2023

This study aimed to analyze potential biomarkers for systemic sclerosis (SSc) by constructing lncRNA-miRNA-mRNA networks in circulating exosomes (cirexos). Differentially expressed mRNAs (DEmRNAs) and lncRNAs (DElncRNAs) SSc cirexos were screened using high-throughput sequencing detected with real-time quantitative PCR (RT-qPCR). genes (DEGs) analyzed the DisGeNET, GeneCards, GSEA4.2.3, Gene Ontology (GO), Kyoto Encyclopedia of Genes Genomes (KEGG) databases. Receiver operating characteristic (ROC) curves, correlation analyses, a double-luciferase reporter gene detection assay used competing endogenous RNA (ceRNA) clinical data. In this study, 286 DEmRNAs 192 DElncRNAs screened, which 18 DEGs same as SSc-related genes. The main pathways included extracellular matrix (ECM) receptor interaction, local adhesion, platelet activation, IgA production intestinal immune network. A hub gene, COL1A1, was obtained protein-protein interaction (PPI) Four ceRNA predicted through Cytoscape. relative expression levels ENST0000313807, NON-HSAT194388.1 significantly higher SSc, while hsa-miR-29a-3p, hsa-miR-29b-3p, hsa-miR-29c-3p lower (P < 0.05). ROC curve showed that ENST00000313807-hsa-miR-29a-3p-COL1A1 network combined biomarker is more valuable than independent diagnosis, it correlated high-resolution CT (HRCT), Scl-70, C-reactive protein (CRP), Ro-52, IL-10, IgM, lymphocyte percentage, neutrophil albumin divided globulin, urea, RDW-SD Double-luciferase ENST00000313807 interacts COL1A1. plasma represents diagnosis treatment SSc.

Language: Английский

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The B-cells paradigm in Systemic Sclerosis: an update on pathophysiology and B-cell targeted therapies

Clinical & Experimental Immunology, Journal Year: 2024, Volume and Issue: unknown

Published: Nov. 5, 2024

Abstract Systemic sclerosis (SSc) is considered a rare autoimmune disease in which there are alterations of both the innate and adaptive immune response resulting production autoantibodies. Abnormalities system compromise normal function blood vessels leading to vasculopathy manifested by Raynaud’s phenomenon, an early sign SSc . As consequence this reactive picture, can evolve tissue fibrosis. Several SSc-specific autoantibodies currently known associated with specific clinical manifestations prognosis. Although pathogenetic role these still unclear, their B cells plasma suggests importance development SSc. This review narratively examines B-cell dysfunctions pathogenesis discusses B-cell-targeted therapies used or potentially useful for management end-organ complications.

Language: Английский

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The Microbiome in Systemic Sclerosis: Pathophysiology and Therapeutic Potential

International Journal of Molecular Sciences, Journal Year: 2022, Volume and Issue: 23(24), P. 16154 - 16154

Published: Dec. 18, 2022

Systemic sclerosis (SSc), also known as scleroderma, is an autoimmune disease with unknown etiology characterized by multi-organ fibrosis. Despite substantial investigation on SSc-related cellular and molecular mechanisms, effective therapies are still lacking. The skin, lungs, gut the most affected organs in SSc, which act physical barriers constantly communicate colonized microbiota. Recent reports have documented a unique microbiome signature, may be pathogenic trigger or driver of SSc. Since microbiota influences efficacy toxicity oral drugs, evaluating drug–microbiota interactions has become area interest treatment. existing evidence highlights potential microbial challenge novel therapeutic option In this review, we summarized current knowledge about mechanisms SSc highlighted underlying role pathogenesis. We discussed latest interventions using microbiomes including animal models. This review aims to elucidate pathophysiological connection Insights into will significantly improve our understanding etiopathogenesis developing therapeutics for

Language: Английский

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Systemic Sclerosis and Atherosclerosis: Potential Cellular Biomarkers and Mechanisms

Frontiers in Bioscience-Scholar, Journal Year: 2023, Volume and Issue: 15(4), P. 16 - 16

Published: Dec. 26, 2023

Systemic sclerosis (SSc) is a rare systemic autoimmune disease of unknown etiology, which characterized by endothelial dysfunction, pathologic vasculopathy, and increased tissue fibrosis. Traditionally, SSc has been regarded as prototypical fibrotic in the family diseases. emphasis placed on three components pathogenesis SSc: vascular, immune, mesenchymal. Microvascular lesions, including dysfunction smooth muscle cell migration into intima vessels SSc, resemble atherosclerotic process. Although microvascular hallmark understanding role vascular lesions patients with remains limited. It still whether cardiovascular risk related to specific cardiac complications (such myocardial fibrosis) or accelerated development atherosclerosis. Different immune types appear be involved immunopathogenesis via activation other cells, fibrosis, damage. Macrophages, B T dendritic neutrophils, cells have reported play most important In our article, we reviewed significant recent studies pathogenetic links between

Language: Английский

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Role of rituximab in the treatment of systemic sclerosis: A literature review

Modern Rheumatology, Journal Year: 2023, Volume and Issue: 33(6), P. 1068 - 1077

Published: April 13, 2023

ABSTRACT This literature review aimed to evaluate the effectiveness of rituximab (RTX) in patients with systemic sclerosis (SSc). PubMed was searched for articles, published through 31 March 2022, on any controlled studies using RTX treatment SSc. Of 85 identified 9 were selected by title/abstract screening and full text examination. All nine articles reported outcomes forced vital capacity (%FVC), seven those modified Rodnan skin scores (mRSS). The results showed that among evaluating lesions SSc, four a significant improvement mRSS when compared control group, whereas three no effect. Among lung lesions, five %FVC In conclusion, may be effective profiles SSc whom indicated unclear, although diffuse cutaneous positive anti-topoisomerase I antibody considered potential targets. Additional are needed assess long-term

Language: Английский

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