Systemic Sclerosis and Related Diseases

Development of a multi-organ single-cell atlas of systemic sclerosis reveals shared and tissue-specific pathogenic pathways.

This study creates a multi-organ single-cell atlas of systemic sclerosis to identify shared and tissue-specific transcriptional programs and potential therapeutic targets.

Spatial proteomic profiling of cardiac stromal populations and microenvironments in systemic sclerosis-associated primary myocardial involvement

The paper uses spatial proteomic profiling to analyze cardiac stromal populations and microenvironments in systemic sclerosis-associated myocarditis, identifying distinct fibroblast subtypes and their spatial interactions.

A mast cell receptor is associated with fibrosis and itch in systemic sclerosis

This study identifies Mrgprb2 as a mast cell receptor involved in fibrosis and itch in systemic sclerosis using a mouse model and human tissue analysis.

Conserved Fibroblast Subsets Drive Shared Fibrotic Mechanisms in Osteoarthritis Synovium and Systemic Sclerosis Skin

This study identifies conserved fibroblast subsets and fibrotic mechanisms in osteoarthritis and systemic sclerosis using single-cell RNA-seq and spatial transcriptomics.

Human skin fibrosis with iPSC-derived organoids reveals RUNX2-mediated fibroblast reprogramming

This study uses human iPSC-derived skin organoids to model fibrosis, identifies RUNX2 as a key driver, and finds a small molecule that suppresses fibrotic programs.

Pharmacologic METTL3 inhibition attenuates TGF-beta-driven profibrotic signature in systemic sclerosis

This study identifies an m6A epitranscriptomic signature in systemic sclerosis that drives profibrotic changes, which can be reversed by METTL3 inhibition.

Longitudinal single-cell modeling reveals monocyte reprogramming in juvenile systemic sclerosis following autologous stem cell transplantation

This study uses longitudinal single-cell data to show changes in monocyte gene expression and metabolic state after ASCT in jSSc patients.

Imaging guided single-cell multiomics unveils shared autoreactive CD4+ T-cell responses in blood, locoregional lymph node and affected tissues of patients with systemic autoimmunity

The paper uses imaging-guided single-cell multiomics to identify shared autoreactive CD4+ T-cell responses in blood, lymph nodes, and tissues of patients with systemic autoimmune diseases.

Compartmental Profiling of PDE4B in Systemic Sclerosis

This study characterizes PDE4B expression in systemic sclerosis (SSc) tissues, showing dysregulation in immune cells and protein enrichment in activated fibroblasts, supporting PDE4B as a therapeutic target.

CCN3-derived peptide BLR-200 impairs YAP activation and attenuates bleomycin-induced skin fibrosis through blocking the generation of Sfrp2-positive fibroblasts

This paper identifies BLR-200, a CCN3-derived peptide, as an anti-fibrotic agent that impairs YAP activation and reduces bleomycin-induced skin fibrosis by blocking Sfrp2-positive fibroblast generation.

Loss of CD109 Amplifies NF-κB Signaling and Inflammatory Reprogramming in Dermal Fibroblasts

This study shows that loss of CD109 enhances NF-κB signaling and inflammatory reprogramming in dermal fibroblasts, contributing to skin inflammation in systemic sclerosis.

Synovitis in systemic sclerosis is an interferon-driven stromal condition distinct from rheumatoid arthritis

This study identifies SSc synovitis as an IFN-driven stromal condition distinct from RA, with implications for IFNAR1 blockade as a therapeutic strategy.

Spatial Orchestration of Skin Fibrosis by a CD8+ T cell-Myofibroblast Axis

The paper identifies CD8+ T cells as key drivers of fibrosis in morphea-spectrum diseases through fibroblast IFN-γ signaling using spatial transcriptomics.

The lncRNA HOTAIR/EZH2 interaction inhibitor AC1Q3QWB (AQB) attenuates fibrotic SSc skin tissue re-modelling

This paper shows that inhibiting the HOTAIR/EZH2 interaction with AQB reduces pro-fibrotic gene expression and tissue remodeling in SSc fibroblasts and skin.

Proteome wide serology reveals immune defined subtypes of gastrointestinal disease in systemic sclerosis

This study identifies immune-defined subtypes of gastrointestinal disease in systemic sclerosis using proteome-wide serology, revealing distinct autoantibody profiles and clinically meaningful disease phenotypes.

Deep immune profiling of the peripheral blood reveals disease- and sex-associated immune cell signatures in patients with systemic sclerosis

The paper identifies sex-associated immune cell signatures in systemic sclerosis patients using high-dimensional immune profiling of peripheral blood mononuclear cells.

Sex-biased Fibroblast Subpopulations and Transcriptional Programs Reveal Mechanisms of Skin Lesion Development in Systemic Sclerosis

The paper identifies sex-biased fibroblast subpopulations and transcriptional programs in systemic sclerosis, revealing sex-specific mechanisms of skin fibrosis.

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.

Blood-to-tissue translation in autoimmune disease: paired single-cell evidence from systemic sclerosis

This study shows that peripheral blood can reflect tissue-resident molecular states in systemic sclerosis through paired single-cell RNA-sequencing, with strong recovery of fibroblast subtype programs.

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.

Patient-derived vascularized skin organoids unravel the role of systemic sclerosis fibroblasts in microvascular dysfunction.

This study uses patient-derived vascularized skin organoids to show that SSc fibroblasts drive microvascular dysfunction through secretome-induced fibrotic responses and crosstalk with endothelial cells.

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.

Transcriptional landscape of CD4+ T cells in Systemic Sclerosis

This study uses scRNA-seq to characterize CD4+ T cell subpopulations in SSc patients, identifying interferon-driven activation, reduced TNF signaling, and altered Treg states.

Clonal autoantibodies identify microbial antigen as trigger of autoreactive B cells in systemic sclerosis

The paper identifies microbial antigen, specifically fungal topoisomerase 1, as a trigger for autoreactive B cells in systemic sclerosis by showing cross-reactivity between human and microbial TOP1.

Imbalance of Ciliary Programs Drives Fibroblast Differentiation and Fibrotic Signaling in Systemic Sclerosis

This study identifies ciliary program imbalance as a driver of fibroblast-to-myofibroblast reprogramming in systemic sclerosis, suggesting ciliotherapy as a potential treatment.

A PERK/FOXO1 AXIS LINKS DNA DAMAGE TO FIBROBLAST SURVIVAL IN DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS

This paper identifies a PERK/FOXO1 axis that enables survival of fibroblasts in diffuse cutaneous systemic sclerosis despite DNA damage, and shows that targeting this axis restores apoptosis.

MONOCYTES STRONGLY INDUCE (MYO)FIBROBLAST CONTRACTION IN A NEW 3D SKIN MODEL TO UNDERSTAND THE INFLAMMATION-FIBROSIS AXIS IN SYSTEMIC SCLEROSIS

This study uses a 3D skin model to show that monocytes and macrophages induce myofibroblast contraction via JAK/STAT3 and TGF-β signaling in systemic sclerosis.

Targeting activated IL-23 signaling in Scleroderma by tildrakizumab

This study investigates the role of IL-23 in systemic sclerosis (SSc) and shows that tildrakizumab reduces fibrosis in experimental models.

The altered T cell landscape in Systemic Sclerosis patients is characterized by dysfunctional type 1 immunity.

This study identifies dysfunctional type 1 immunity in SSc patients through altered T cell subsets and reduced IFN-γ production.