ArticlePulmonary circulation
Clinical trial protocol for TRANSFORM-UK: A therapeutic open-label study of tocilizumab in the treatment of pulmonary arterial hypertension.
Article in Pulmonary circulation. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 51 papers.
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Who cites it
51 citing papers in PubMed, 80 citations in OpenAlex.
- Integrated Metabolic and Immune Molecular Subtyping and a Molecular Classification Score for Revealing Disease Heterogeneity in Pulmonary Arterial Hypertension.Metabolites · 2026Article
- Paradigm shift: from pulmonary vasodilation to cardiopulmonary dual-track therapy-a comprehensive review of pathophysiology and advances in pulmonary hypertension-associated right heart failure.Respiratory research · 2026Review
- Advances in Novel Therapeutic Strategies for Pulmonary Arterial Hypertension.Canadian respiratory journal · 2026Review
- C-C Motif chemokine receptor-2 blockade ameliorates pulmonary hypertension in rats and synergizes with a pulmonary vasodilator.Cardiovascular research · 2025Article
- Current Overview of the Biology and Pharmacology in Sugen/Hypoxia-Induced Pulmonary Hypertension in Rats.Journal of aerosol medicine and pulmonary drug delivery · 2024Review
- Tetralogy of Fallot Across the Lifespan: A Focus on the Right Ventricle.CJC pediatric and congenital heart disease · 2023Review
- Identification of immune-associated signatures and potential therapeutic targets for pulmonary arterial hypertension.Journal of cellular and molecular medicine · 2023Article
- Interleukin-6 and pulmonary hypertension: from physiopathology to therapy.Frontiers in immunology · 2023Review
- Review
- Recent Advances in the Treatment of Pulmonary Arterial Hypertension.Pharmaceuticals (Basel, Switzerland) · 2022Review
- Article
- Cytokine profiling in pulmonary arterial hypertension: the role of redox homeostasis and sex.Translational research : the journal of laboratory and clinical medicine · 2022Article
- Kynurenine metabolites predict survival in pulmonary arterial hypertension: A role for IL-6/IL-6Rα.Scientific reports · 2022Article
- Future Perspectives of Pulmonary Hypertension Treatment.Acta Cardiologica Sinica · 2022Review
- Mendelian randomisation and experimental medicine approaches to interleukin-6 as a drug target in pulmonary arterial hypertension.The European respiratory journal · 2022Article
- Pulmonary hypertension: Linking inflammation and pulmonary arterial stiffening.Frontiers in immunology · 2022Review
- Pulmonary Arterial Hypertension: Emerging Principles of Precision Medicine across Basic Science to Clinical Practice.Reviews in cardiovascular medicine · 2022Article
- Bioinformatic exploration of the immune related molecular mechanism underlying pulmonary arterial hypertension.Bioengineered · 2021Article
- Approaches to treat pulmonary arterial hypertension by targeting BMPR2: from cell membrane to nucleus.Cardiovascular research · 2021Review
- Treatment of pulmonary arterial hypertension in children.Cardiovascular diagnosis and therapy · 2021Review
Corrections and comments
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Authors and funding
11 authors at 6 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Our aim is to assess the safety and potential efficacy of a novel treatment paradigm in pulmonary arterial hypertension (PAH), immunomodulation by blocking interleukin-6 (IL6) signaling with the IL6 receptor antagonist, tocilizumab. Inflammation and autoimmunity are established as important in PAH pathophysiology. One of the most robust observations across multiple cohorts in PAH has been an increase in IL6, both in the lung and systemically. Tocilizumab is an IL-6 receptor antagonist established as safe and effective, primarily in rheumatoid arthritis, and has shown promise in scleroderma. In case reports where the underlying cause of PAH is an inflammatory process such as systemic lupus erythematosus, mixed connective tissue disease (MCTD), and Castleman's disease, there have been case reports of regression of PAH with tocilizumab. TRANSFORM-UK is an open-label study of intravenous (IV) tocilizumab in patients with group 1 PAH. The co-primary outcome measures will be safety and the change in resting pulmonary vascular resistance (PVR). Clinically relevant secondary outcome measurements include 6-minute walk distance, WHO functional class, quality of life score, and N-terminal pro-brain natriuretic peptide (NT-proBNP). If the data support a potentially useful therapeutic effect with an acceptable risk profile, the study will be used to power a Phase III study to properly address efficacy.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.