O. Distler

Radiomic analysis of high-resolution computed tomography predicts interstitial lung disease progression and mortality in systemic sclerosis

Abstract Objective The automated assessment of chest high-resolution CT (HRCT) scans allows the objective quantification of interstitial lung disease (ILD)–related features. Here, we explored the ability of HRCT-derived radiomic features to predict all-cause mortality and ILD progression in SSc-ILD patients. Methods We...

Comparison of Culture Systems for Mouse Living Myocardial Slices in Cardiac Fibrosis Research

Conceptual framework for the definition of disease modification in systemic sclerosis and potential implications for drug development and clinical study design.

Systemic sclerosis (SSc) is a multifaceted systemic autoimmune disease, characterised by the interplay of vascular alterations, immune dysregulation, and fibrosis. The molecular changes and the clinical presentation of SSc are highly heterogeneous and vary considerably between individual patients. This heterogeneity ha...

Dysferlin is a novel regulator of COMP-positive matrifibrocytes in heart failure

Dysferlin is a stress-inducible antifibrotic regulator that limits maladaptive fibroblast differentiation and myocardial fibrosis, thereby representing a potential therapeutic target to attenuate adverse cardiac remodelling in HF.

Demographic and clinical features of systemic sclerosis patients with anti-U1RNP antibodies: an European Scleroderma Trials and Research (EUSTAR) analysis

To investigate the demographic and clinical features of systemic sclerosis (SSc) patients with anti-U1RNP antibodies using the European Scleroderma Trials and Research (EUSTAR) database, a phenotype less well-defined in SSc than other more commonly associated antibodies. SSc patients fulfilling classificat...

The dual PPAR-α/δ agonist elafibranor attenuates TGF-β1-induced cardiac fibrosis through redox-metabolic and bioenergetic reprogramming in human cardiac models

Elafibranor mitigates TGF-β1-driven cardiac fibrosis by suppressing fibroblast activation and ECM remodelling while promoting adaptive metabolic, redox, and bioenergetic responses, supporting balanced PPAR-α/δ activation as a potential therapeutic strategy for cardiac fibrosis.

Lanifibranor (IVA-337) - a pan-PPAR agonist suppresses TGF-β1-induced cardiac fibrosis and rescues cardiomyocyte function

Lanifibranor attenuates TGF-β1-driven cardiac fibrosis by combining antifibrotic effects with metabolic and functional improvements in human models.

The Role of Inflammation in the Pathogenesis of Pulmonary Fibrosis: Mechanisms and Treatment Implications.

Insights from experimental models of pulmonary fibrosis and translational research have significantly advanced understanding of these processes and are reviewed in this article, together with current therapeutic advances and future directions.