Systemic Sclerosis and Related Diseases

Aurora A kinase activation contributes to the fibrotic phenotype in Systemic Sclerosis through primary cilia shortening

This study identifies AURKA/HDAC6 activation as a driver of primary cilia shortening and fibroblast activation in systemic sclerosis.

Enhanced recognition of topoisomerase 1 upon DNA binding by a subset of anti-topoisomerase 1 autoantibodies in systemic sclerosis

This study shows that some anti-topoisomerase 1 autoantibodies (ATAs) in systemic sclerosis (SSc) bind more strongly to TOP1-DNA cleavage complexes, correlating with disease severity and immune activation.

Neutrophil Reprogramming Underlie Vasculopathy and Lung Disease in Systemic Sclerosis

This study identifies neutrophil reprogramming in systemic sclerosis (SSc) driven by HMGB1+ extracellular vesicles, leading to lung inflammation and fibrosis, and suggests therapeutic targeting of HMGB1.

Human blood vessel organoids recapitulate key mechanisms of transition from vasculopathy to fibrosis in systemic sclerosis

This study uses blood vessel organoids to model systemic sclerosis vasculopathy and fibrosis, showing that SSc serum induces epigenetic and molecular changes linked to endothelial dysfunction and potential therapeutic targets.

Targeting Tenascin-C-Toll-like Receptor 4 signalling with Adhiron-derived small molecules - a viable strategy for reducing fibrosis in Systemic Sclerosis

This paper identifies small molecules targeting the Tenascin C fibrinogen-like globe domain to reduce fibrosis in systemic sclerosis by inhibiting Toll-like receptor 4 signaling.

Spatially informed phenotyping by cyclic-in-situ-hybridization identifies novel fibroblast populations and their pathogenic niches in systemic sclerosis

The paper uses spatial transcriptomic methods to identify novel fibroblast subpopulations in systemic sclerosis skin and their pathogenic roles.

Distinct cAMP regulation in scleroderma lung and skin myofibroblasts governs their dedifferentiation via p38α inhibition

This study compares cAMP regulation and dedifferentiation in lung and skin myofibroblasts from scleroderma patients, finding tissue-specific differences in PGE2 response and p38 inhibition effects.

Distinct metabolic profiles of circulating plasmacytoid dendritic cells in systemic sclerosis patients stratified by clinical phenotypes

The paper examines metabolic profiles of circulating plasmacytoid dendritic cells (pDCs) in systemic sclerosis (SSc) patients, finding differences based on clinical phenotypes.

Gene Expression Meta-Analysis Reveals Aging and Cellular Senescence Signatures in Scleroderma-associated Interstitial Lung Disease

This study identifies aging and senescence signatures in SSc-ILD through gene expression analysis and telomere length measurement, linking them to disease pathology.

Loss of Protein Kinase D2 Activity Protects Against Bleomycin-induced Dermal Fibrosis in Mice

This study shows that reducing PKD2 activity in mice prevents bleomycin-induced dermal fibrosis by decreasing collagen and inflammatory markers.

RNA-Seq analysis identifies novel roles for the primary cilia gene SPAG17 and the SOX9 locus non-coding RNAs in systemic sclerosis

The paper uses RNA-Seq to analyze SSc patient and control samples, identifying SPAG17 and SOX9 non-coding RNAs as novel factors in SSc pathogenesis.

A critical role of AREG for bleomycin-induced skin fibrosis

The paper identifies AREG as a critical driver of bleomycin-induced skin fibrosis and shows that targeting the AREG-EGFR-MEK pathway can prevent fibrosis.

Differential DNA methylation landscape in skin fibroblasts from African Americans with systemic sclerosis

The paper identifies differential DNA methylation in skin fibroblasts from African American systemic sclerosis patients, linking it to immune and metabolic pathways.

Genetic interactions affect lung function in patients with systemic sclerosis

This study identifies a three-gene network influencing lung function and autoantibody status in systemic sclerosis patients through genetic interaction analysis.

Evaluation of the antifibrotic potency by knocking down SPARC, CCR2 and SMAD3

The paper evaluates the antifibrotic effects of triple siRNA targeting SPARC, CCR2, and SMAD3 in a mouse model of fibrosis, showing reduced inflammation and fibrosis through multi-target intervention.

A functional genomic meta-analysis of clinical trials in systemic sclerosis: towards precision medicine and combination therapy

The paper performs a meta-analysis of gene expression data from SSc clinical trials to identify therapeutic targets and predict treatment response using machine learning.