Zephyrus II Study Shows Potential for IPF Treatment

Work leading up to Zephyrus II: A Phase 3 Randomized, Double-Blind, Placebo-Controlled Efficacy and Safety Study of Pamrevlumab in Subjects with Idiopathic Pulmonary Fibrosis (IPF)



Idiopathic pulmonary fibrosis (IPF) is a chronic, fatal lung disease of unknown cause. In people with IPF, the tiny air sacs in the lung (alveoli) become damaged and increasingly fibrotic (scarred). Currently, there are limited treatment options that only slow down disease progression. These drugs also have significant side effects that can impact treatment compliance. Thus, a need exists for new and improved pharmacological therapies for IPF.

Pamrevlumab (FG-3019) is an antibody against connective tissue growth factor (CTGF), which has been shown be to be an important factor in the process of fibrosis. CTGF is thought to interact with key factors of fibrosis. In pre-clinical models of fibrosis, stimulation of the CTGF gene promoted a fibrotic response. Furthermore, in patients with IPF, CTGF gene activity was found to be higher on lung biopsy compared to non-IPF patients. These observations suggest that CTGF may have a role in the pathogenesis of IPF. This has led to promising work in clinical trials.

Phase 2 study:

The phase 2 ‘PRAISE’ study is the most recently completed trial designed to assess the efficacy and safety profile of Pamrevlumab in IPF patients. The study was conducted across 39 medical centres in seven countries, between 2013 and 2017. Identified participants were randomised into a 48 week treatment period, with either Pamrevlumab or placebo (as a control).  The safety and tolerability of Pamrevlumab were assessed very closely, alongside spirometry and CT scans to review the efficacy of the drug.


Overall, Pamrevlumab reduced the decline in FVC (the amount of air that can be forcibly exhaled from your lungs after taking the deepest breath possible) by 60% at week 48. Fewer patients showed disease progression on Pamrevlumab than the placebo group. Moreover, Pamrevlumab was well tolerated, with a safety profile similar to that of placebo.


Pamrevlumab reduced the progression of IPF and was safe and well tolerated.

Current and future work:

Now in phase 3 development with larger group testing, the ‘Zephyrus II’ study is open for recruitment at UCLH. With this important work, Pamrevlumab shows a promising role in the future of IPF treatments.  

If you have pulmonary fibrosis, are a patient of UCLH and are interested in joining this study, please speak to your specialist or email our research team on uclh.respiratory.research.nurses@nhs.net


Written by Dr Sharenja Ratnakumar, Clinical Fellow / February 2023


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