Dr. Chris Brereton outlined current and upcoming research trials in interstitial lung disease (ILD) being conducted in Wessex. Current trials included EME-TIPAC studying the efficacy of co-trimoxazole in IPF patients, MA39189 studying pirfenidone in unclassified fibrosis, and an exercise feasibility study in IPF patients. Upcoming trials included Zephyr studying GBT-440 in hypoxemic IPF patients, Celegene evaluating CC-90001 in IPF, IPF JES measuring asbestos exposure in IPF patients, and TRAIL1 studying pirfenidone in rheumatoid arthritis-ILD. Dr. Brereton provided details on eligibility criteria and study

1. ILD research in Wessex
WILD meeting 9th October 2017
Dr Chris Brereton

3. Outline
• Current research trials
– EME-TIPAC (IPF)
– MA39189 (unclassified fibrosis)
– Exercise in IPF
• Upcoming research trials
– Zephyr (IPF)
– Celegene (IPF)
– IPF JES
– TRAIL1 (rheumatoid arthritis-ILD)
– Time to diagnosis in IPF

4. EME-TIPAC
• Efficacy and Mechanism Evaluation of Treating
IPF with addition of Co-trimoxazole
• Co-trimoxazole (septran) a broad spectrum
antibiotic.
• Eligible patients
– Moderate to severe IPF
– CAN be on other treatments
(nintedanib/pirfenidone)

5. EME-TIPAC
• Participants are randomised to drug or placebo.
• Undertake treatment for between 12 and 42
months.
• Hospital visits at 6 weeks, 3 months, 6 months, 9
months and then every 6 months
• Clinical questionnaires, bloods tests and lung
function.
• 6 participants in Wessex so far. Need another
two.

6. MA39189
• Pirfenidone in patients with unclassifiable
progressive pulmonary fibrosis
• Randomised to drug versus placebo and
treated for 24 weeks (total study treatment 91
weeks including open label safety follow up)
• 10 hospital visits (plus more if moving to open
label follow up)
• Looking for at least 2 participants

7. Exercise in IPF
• 8 week exercise program for patients with
mild to moderate IPF (feasibility study)
• Structured cycling-based training program
tailored individually for each patient’s fitness
level
• Also involves blood tests, lung function,
questionnaires

8. Exercise in IPF
• Looking for 6 participants
• Training at UHS (40 minutes, twice weekly for
8 weeks)
• Car parking and petrol can be reimbursed

10. Zephyr
• GBT-440 on hypoxaemia in subjects with IPF
using supplemental oxygen at rest
• Eligible participants
– Requiring supplemental oxygen at rest
– Able to walk 100 metres
– CAN be on pirfenidone/nintedanib
• All patients will receive the drug (no placebo)

11. Zephyr
• 8 hospital visits over 151 days
• Patients will need to undergo lung function
tests, 6 minute walk tests, blood tests and
questionnaires
• Also oxygen titration tests and arterial blood
gas.

12. TRAIL1
• Tolerability and efficacy of pirfenidone in
rheumatoid arthritis ILD
• Randomised to drug versus placebo
• Study duration 15 months (treatment for 12
months)
• Looking for 6-8 participants
• Eligible participants will have 8 hospital visits,
a CT chest, lung function, blood tests

13. IPF-JES
• IPF Job exposure study
• Looking to measure asbestos exposure in
participants diagnosed with IPF (i.e.
Questionairre over the phone)
• Also looking at genetic interactions with
asbestos (i.e. Blood test)
• Eligible patients – not worked outside UK for
more than 1 year
• Looking for 30 recruits.

15. Celegene
• Evaluating CC-90001 in IPF
• 24 weeks randomised to drug (two different
doses) versus placebo
• Followed by 28 week treatment extension of
blinded study drug
• Between 9 and 15 hospital visits
• Eligible participants
– Mild-moderate IPF
– NOT on pirfenidone/nintedanib

16. Time to diagnosis and treatment in IPF
• Measuring the time between referral by GP
and final diagnosis/treatment
• Comparing this length of time to outcomes
• De-identification and review of medical
records for all patients diagnosed with IPF in
past 5 years

18. Some final points