Jörg H. W. Distler
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View article: Distinct myeloid precursors and their interaction with mesenchymal cells orchestrate the spreading of psoriatic disease from the skin to the joints
Distinct myeloid precursors and their interaction with mesenchymal cells orchestrate the spreading of psoriatic disease from the skin to the joints Open
Psoriatic disease initially affects the skin, but later extends to the joints. Herein, we describe a two-step process that orchestrates spreading of inflammation from the skin to the joints. Induction of psoriatic skin disease in photoconv…
View article: Performance and Practical Considerations of Large and Small Language Models in Clinical Decision Support in Rheumatology
Performance and Practical Considerations of Large and Small Language Models in Clinical Decision Support in Rheumatology Open
Large language models (LLMs) show promise for supporting clinical decision-making in complex fields such as rheumatology. Our evaluation shows that smaller language models (SLMs), combined with retrieval-augmented generation (RAG), achieve…
View article: Spatially informed phenotyping by cyclic-in-situ-hybridisation identifies novel fibroblast populations and their pathogenic niches in systemic sclerosis
Spatially informed phenotyping by cyclic-in-situ-hybridisation identifies novel fibroblast populations and their pathogenic niches in systemic sclerosis Open
Our cISH-based, spatially resolved transcriptomic approach identified novel fibroblast subpopulations deregulated in SSc skin with specific pathogenic roles. COL8A1+ FBs and their immune interactions may also have potential as biomarkers f…
View article: Matrix stiffness regulates profibrotic fibroblast differentiation and fibrotic niche activation in systemic sclerosis
Matrix stiffness regulates profibrotic fibroblast differentiation and fibrotic niche activation in systemic sclerosis Open
Our findings suggest that increased matrix stiffness promotes fibroblast precursor differentiation into SFRP2+ COMP+ PU.1+ myofibroblasts, thereby sustaining the vicious cycle of persistent fibrosis in abse…
View article: Lung function and skin fibrosis changes as predictors of survival in SSc-associated interstitial lung disease: a EUSTAR study
Lung function and skin fibrosis changes as predictors of survival in SSc-associated interstitial lung disease: a EUSTAR study Open
Objectives This study assessed how changes in lung function, skin fibrosis and digital ulceration (DU) burden predict mortality in patients with SSc-associated interstitial lung disease (SSc-ILD), the leading cause of death in SSc. Methods…
View article: POS0249 SPATIAL PROTEOMIC-BASED PHENOTYPING OF FIBROBLAST SUBPOPULATIONS AND THEIR MICROENVIRONMENT IN SYSTEMIC SCLEROSIS PRIMARY HEART INVOLVEMENT
POS0249 SPATIAL PROTEOMIC-BASED PHENOTYPING OF FIBROBLAST SUBPOPULATIONS AND THEIR MICROENVIRONMENT IN SYSTEMIC SCLEROSIS PRIMARY HEART INVOLVEMENT Open
View article: POS0946 IMMUNOCLEANSING-ASSOCIATED TOXICITY SYNDROME (ICATS) IN CAR T-CELL TREATED PATIENTS WITH AUTOIMMUNE DISEASE
POS0946 IMMUNOCLEANSING-ASSOCIATED TOXICITY SYNDROME (ICATS) IN CAR T-CELL TREATED PATIENTS WITH AUTOIMMUNE DISEASE Open
View article: POS0003 Specific targeting of the 5-hydroxytryptamine 2B receptor (5-HT2BR) demonstrates antifibrotic effects in murine models and ex vivo models of scleroderma skin
POS0003 Specific targeting of the 5-hydroxytryptamine 2B receptor (5-HT2BR) demonstrates antifibrotic effects in murine models and ex vivo models of scleroderma skin Open
View article: POS0252 IDENTIFICATION OF FIBROBLAST POPULATIONS AND THEIR PATHOGENIC NICHES IN SYSTEMIC SCLEROSIS BY IMAGING-BASED SPATIAL TRANSCRIPTOMICS
POS0252 IDENTIFICATION OF FIBROBLAST POPULATIONS AND THEIR PATHOGENIC NICHES IN SYSTEMIC SCLEROSIS BY IMAGING-BASED SPATIAL TRANSCRIPTOMICS Open
View article: OP0011 In vitro modelling of vasculopathy in systemic sclerosis by human blood vessel organoids
OP0011 In vitro modelling of vasculopathy in systemic sclerosis by human blood vessel organoids Open
View article: Persisting CD19.CAR-T cells in combination with nintedanib: clinical response in a patient with systemic sclerosis-associated pulmonary fibrosis after 2 years
Persisting CD19.CAR-T cells in combination with nintedanib: clinical response in a patient with systemic sclerosis-associated pulmonary fibrosis after 2 years Open
View article: Spatial multiomics decipher fibroblast–macrophage dynamics in systemic sclerosis
Spatial multiomics decipher fibroblast–macrophage dynamics in systemic sclerosis Open
Our spatially resolved atlas uncovered dynamic fibroblast-macrophage interplay as a hallmark of fibrotic niche expansion. These findings offer spatiotemporal insights into disease mechanisms and pave the way for advanced mechanistic and th…
View article: Acidosis as a promising early indicator of mortality among point-of-care parameters and vital signs in non-traumatic critically ill patients
Acidosis as a promising early indicator of mortality among point-of-care parameters and vital signs in non-traumatic critically ill patients Open
View article: Local immune effector cell-associated toxicity syndrome in CAR T-cell treated patients with autoimmune disease: an observational study
Local immune effector cell-associated toxicity syndrome in CAR T-cell treated patients with autoimmune disease: an observational study Open
Deutsche Forschungsgemeinschaft (DFG), German Cancer Aid, Bundesministerium für Bildung und Forschung, European Union, Staedtler Foundation, Lupus Research Alliance, and donations from the Bendel family and the Bleyl family.
View article: Single cell mapping of the metabolic landscape of skin fibrosis in systemic sclerosis
Single cell mapping of the metabolic landscape of skin fibrosis in systemic sclerosis Open
Tissue resident cells undergo metabolic reprogramming during fibrotic tissue remodeling to meet their changing metabolic demands required for extracellular matrix production and phenotypic transitions in fibrosis. However, the metabolic re…
View article: Human blood vessel organoids recapitulate key mechanisms of transition from vasculopathy to fibrosis in systemic sclerosis
Human blood vessel organoids recapitulate key mechanisms of transition from vasculopathy to fibrosis in systemic sclerosis Open
Summary Systemic sclerosis (SSc) is an autoimmune disease that transitions from vasculopathy as an initiating pathogenic event to tissue fibrosis. The mechanisms of these transitions remain, however, poorly understood, mainly because compl…
View article: Publisher Correction: Fibrosis: cross-organ biology and pathways to development of innovative drugs
Publisher Correction: Fibrosis: cross-organ biology and pathways to development of innovative drugs Open
View article: Diffuse Hyperpigmentierung bei systemischer Sklerose
Diffuse Hyperpigmentierung bei systemischer Sklerose Open
Zusammenfassung Pigmentstörungen sind ein häufig beobachtetes Phänomen bei Patienten mit systemischer Sklerose (SSc). Sie können bei bis zu 50 % der Betroffenen auftreten und umfassen sowohl Hypopigmentierungen als auch diffuse Hyperpigmen…
View article: Antifibrotic effects of specific targeting of the 5‐hydroxytryptamine 2B receptor (5‐HT<sub>2B</sub>R) in murine models and ex vivo models of scleroderma skin
Antifibrotic effects of specific targeting of the 5‐hydroxytryptamine 2B receptor (5‐HT<sub>2B</sub>R) in murine models and ex vivo models of scleroderma skin Open
Objective Systemic sclerosis (SSc) is a connective tissue disease with fibrotic remodeling of the skin and various internal organs. SSc is associated with the highest case‐specific mortality of all rheumatic autoimmune diseases with limite…
View article: Publisher Correction: An international perspective on the future of systemic sclerosis research
Publisher Correction: An international perspective on the future of systemic sclerosis research Open
View article: Evaluation of Systemic Sclerosis Primary Heart Involvement and Chronic Heart Failure in the European Scleroderma Trials and Research Cohort
Evaluation of Systemic Sclerosis Primary Heart Involvement and Chronic Heart Failure in the European Scleroderma Trials and Research Cohort Open
Background Systemic sclerosis (SSc) primary heart involvement (SSc‐pHI) is one of the leading causes of mortality in SSc. We aimed to evaluate risk factors for SSc‐pHI and its progression and the outcomes in the EUSTAR (European Scleroderm…
View article: Avenciguat: a novel soluble guanylate cyclase activator that affects multiple cell types to inhibit IFN-1 signalling and fibrosis
Avenciguat: a novel soluble guanylate cyclase activator that affects multiple cell types to inhibit IFN-1 signalling and fibrosis Open
Objectives The soluble guanylate cyclase (sGC) stimulator riociguat is approved for the treatment of pulmonary arterial hypertension and may have antifibrotic effects. However, in fibrotic tissues, oxidative stress and hypoxia can render s…
View article: Mapping spatially-resolved transcriptomes in systemic sclerosis
Mapping spatially-resolved transcriptomes in systemic sclerosis Open
Systemic sclerosis (SSc) is a prototypical fibrotic disease with high mortality and limited treatment options. Despite advances in single-cell RNA sequencing (scRNA-seq), the comprehensive understanding of cellular heterogeneity and cell-c…
View article: Combination therapies of porcupine inhibition with ruxolitinib, ibrutinib, or belumosudil in murine sclerodermatous GVHD
Combination therapies of porcupine inhibition with ruxolitinib, ibrutinib, or belumosudil in murine sclerodermatous GVHD Open
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View article: Compartment-Specific NK Cell Phenotypes Reveal Distinct Maturation and Activation States in Inflammatory Arthritis
Compartment-Specific NK Cell Phenotypes Reveal Distinct Maturation and Activation States in Inflammatory Arthritis Open
Introduction: Synovial natural killer (NK) cells contribute to inflammation in arthritis by secreting cytokines and modulating synovial fibroblast activation. The aim of this study was to describe systemic versus local inflammatory changes…
View article: Disturbed Spatial <scp>WNT</scp> Activation—A Potential Driver of the Reticularized Skin Phenotype in Systemic Sclerosis
Disturbed Spatial <span>WNT</span> Activation—A Potential Driver of the Reticularized Skin Phenotype in Systemic Sclerosis Open
Objective Little is known on the mechanisms necessary to maintain the physiologic adult human skin integrity. This study aims to quantitatively describe anatomic changes in systemic sclerosis (SSc)–skin compared with controls and investiga…
View article: Case report: Detecting giant cell arteritis in [68Ga]Ga-DOTA-Siglec-9-PET/CT
Case report: Detecting giant cell arteritis in [68Ga]Ga-DOTA-Siglec-9-PET/CT Open
Objectives This study aimed to evaluate the diagnostic utility of [ 68 Ga]Ga-DOTA-Siglec-9 positron emission tomography-computed tomography (PET/CT) in assessing disease activity in a patient experiencing a relapse of giant cell arteritis …
View article: CD63 as novel target for nanoemulsion-based <sup>19</sup>F MRI imaging and drug delivery to activated cardiac fibroblasts
CD63 as novel target for nanoemulsion-based <sup>19</sup>F MRI imaging and drug delivery to activated cardiac fibroblasts Open
Rationale: Cardiac fibroblasts are activated following myocardial infarction (MI) and cardiac fibrosis is a major driver of the growing burden of heart failure. A non-invasive targeting method for activated cardiac fibroblasts would…
View article: miR-3606-3p alleviates skin fibrosis by integratively suppressing the integrin/FAK, p-AKT/p-ERK, and TGF-β signaling cascades
miR-3606-3p alleviates skin fibrosis by integratively suppressing the integrin/FAK, p-AKT/p-ERK, and TGF-β signaling cascades Open
MiR-3606-3p is downregulated in skin fibrosis. Moreover, it negatively correlates with disease severity. Functionally, miR-3606-3p inhibits fibrogenesis, migration, inflammation, and proliferation of fibroblasts. Mechanistically, miR-3606-…
View article: Cure as a treatment target in rheumatoid arthritis and systemic sclerosis—achievable aim or mission impossible? FOREUM stimulates new industry-academia collaboration
Cure as a treatment target in rheumatoid arthritis and systemic sclerosis—achievable aim or mission impossible? FOREUM stimulates new industry-academia collaboration Open
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