ArticleThe European respiratory journal2022
Pirfenidone exacerbates Th2-driven vasculopathy in a mouse model of systemic sclerosis-associated interstitial lung disease.
Article in The European respiratory journal, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 20 citations in OpenAlex.
- Next-Generation Sequencing in Pulmonary Fibrosis: Translational Promise and Current Clinical Limitations.Current issues in molecular biology · 2026Review
- Unlocking multi-institutional insights into disease progression with PEAL as a lossless, one-shot federated learning solution.NPJ digital medicine · 2026Article
- Integrative Perspectives on Pirfenidone: Mechanistic Insights, Pharmacodynamics, and Emerging Therapeutic Strategies for Fibrotic Disorders.AAPS PharmSciTech · 2026Review
- Tolerability and Safety of Nintedanib and Immunosuppression in Autoimmune Inflammatory Myopathy-Associated Progressive Pulmonary Fibrosis.CHEST pulmonary · 2026Article
- Transcriptional signatures of endothelial cells shape immune responses in cardiopulmonary health and disease.JCI insight · 2025Review
- Pulmonary vascular remodeling in Fra-2 transgenic mice is driven by type 2 inflammation and accompanied by pulmonary vascular hyperresponsiveness.American journal of physiology. Lung cellular and molecular physiology · 2025Article
- CLCA1 and BPIFB1 are potential novel biomarkers for asthma: an iTRAQ analysis.Journal of thoracic disease · 2024Article
- Towards a Better Prognosis in Patients with Systemic Sclerosis-Related Pulmonary Arterial Hypertension: Recent Developments and Perspectives.Journal of clinical medicine · 2024Review
- Recent Insights into Cellular and Molecular Mechanisms of Defective Angiogenesis in Systemic Sclerosis.Biomedicines · 2024Review
- The vascular perspective on acute and chronic lung disease.The Journal of clinical investigation · 2023Review
- Potential therapeutic targets of macrophages in inhibiting immune damage and fibrotic processes in musculoskeletal diseases.Frontiers in immunology · 2023Review
- Fra-2 Is a Dominant Negative Regulator of Natural Killer Cell Development.Frontiers in immunology · 2022Article
- The fibrosis puzzle of systemic sclerosis-associated ILD and the quest for targeted interventions.Therapeutic advances in respiratory diseaseReview
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Authors and funding
14 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSystemic sclerosis (SSc) is an autoimmune disease characterised by severe vasculopathy and fibrosis of various organs including the lung. Targeted treatment options for SSc-associated interstitial lung disease (SSc-ILD) are scarce. We assessed the effects of pirfenidone in a mouse model of SSc-ILD.
methodsPulmonary function, inflammation and collagen deposition in response to pirfenidone were assessed in Fra-2-overexpressing transgenic (Fra-2 TG) and bleomycin-treated mice. In Fra-2 TG mice, lung transcriptome was analysed after pirfenidone treatment.
resultsPirfenidone treatment attenuated pulmonary remodelling in the bleomycin model, but aggravated pulmonary inflammation, fibrosis and vascular remodelling in Fra-2 TG mice. Pirfenidone increased interleukin (IL)-4 levels and eosinophil numbers in lung tissue of Fra-2 TG mice without directly affecting eosinophil activation and migration
conclusionThis study shows that antifibrotic properties of pirfenidone may be overruled by unwanted interactions with pre-injured endothelium in a setting of high T-helper type 2 inflammation in a model of SSc-ILD. Careful ILD patient phenotyping may be required to exploit benefits of pirfenidone while avoiding therapy failure and additional lung damage in some patients.
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