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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\u20106 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\u2019s disease, there have been case reports of regression of PAH with tocilizumab. TRANSFORM\u2010UK is an open\u2010label study of intravenous (IV) tocilizumab in patients with group 1\u00a0PAH. The co\u2010primary outcome measures will be safety and the change in resting pulmonary vascular resistance (PVR). Clinically relevant secondary outcome measurements include 6\u2010minute walk distance, WHO functional class, quality of life score, and N\u2010terminal pro\u2010brain natriuretic peptide (NT\u2010proBNP). 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.<\/jats:p>","DOI":"10.1177\/2045893217735820","type":"journal-article","created":{"date-parts":[[2017,9,28]],"date-time":"2017-09-28T11:46:42Z","timestamp":1506599202000},"page":"1-8","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":71,"title":["Clinical trial protocol for TRANSFORM\u2010UK: A therapeutic open\u2010label study of tocilizumab in the treatment of pulmonary arterial hypertension"],"prefix":"10.1002","volume":"8","author":[{"given":"Jules","family":"Hern\u00e1ndez\u2010S\u00e1nchez","sequence":"first","affiliation":[{"name":"Papworth Trials Unit Collaboration Papworth Hospital Cambridge UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louise","family":"Harlow","sequence":"additional","affiliation":[{"name":"Pulmonary 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