Research Update

APF’s vision is to find a cure for pulmonary fibrosis so that everyone affected by the disease has a better future.

Research funded by APF

Action for Pulmonary Fibrosis (APF) is now one of the major charities funding research on pulmonary fibrosis in Europe. With your help, we are determined to make the greatest contribution we can to national and global efforts to find a cure for this devastating disease. Find out how you can raise funds for us.

Every year, APF commits more funds to research into pulmonary fibrosis. In 2019, we put almost £600,000 into research, all of which was donated by our amazing supporters and fundraisers. We would like to give a big thankyou to all of you!

In the UK, Europe and North America, increasing collaboration between doctors, scientists and patient advocacy groups is leading to real improvements in outcomes for patients with this devastating disease. As a charity run jointly by patients and leading clinicians, we are making an important contribution to these efforts.

We are a founder member of the European IPF Federation (EU-IPFF) and maintain close contacts with its Scientific Advisory Group and developments across Europe.

Genetic research

So far, IPF has been shown to be associated with almost 20 genetic variations in patients.

Genetic insights raise the future prospect of precision medicine, making it possible to target specific treatments to patients based on genetic or molecular abnormalities.

Over the next three years, many more IPF-associated genetic variations are expected to be identified as a result of research by Dr Richard Allen at the University of Leicester. This will hopefully lead to the identification of the different chemical and biological pathways involved in the process of fibrosis and to new treatments.

This research is funded by Action for Pulmonary Fibrosis. Help us fund more research like this.

Bacterial causes of pulmonary fibrosis

Another important avenue for research is the role played by bacteria in causing IPF and progress of the disease.

Dr Phil Molyneaux of Brompton Hospital and Imperial College is investigating the role different bacteria play in disease progression and whether taking prophylactic antibiotics can slow down disease progression.  If they do, this could open another possible new treatment for patients.

This research is funded by Action for Pulmonary Fibrosis. Help us fund more research like this.

Other pulmonary fibrosis conditions

There has also been research into treatments and causes for other types of pulmonary fibrosis, including chronic hypersensitivity pneumonitis and autoimmune-related interstitial lung disease (such as rheumatoid arthritis-ILD).

Listening to the patient voice

Action for Pulmonary Fibrosis plays an important role in ensuring that patient views are included in the design and implementation of other major research projects, financed by the National Institute of Health Research (NIHR) and others.

We are involved in this way in two major NIHR studies.

• The first, at the University of Nottingham, seeks to identify biomarkers in the blood, which would make it possible to assess whether a person has rapidly or slowly progressing disease and to tailor their treatment accordingly.

• The second, coordinated by the University of East Anglia, is investigating the potential benefit for IPF patients of controlling gastric reflux. Both these have the potential to transform treatments for patients.

Anti-fibrotic drugs

Two anti-fibrotic drugs for IPF  – pirfenidone and nintedanib – have been approved for use in IPF and more are in the pipeline. If on-going trials of these new therapies prove successful, they could become available to patients in the next two to five years.

Pirfenidone and nintedanib have recently been tested on other progressive forms of pulmonary fibrosis, such as hypersensitivity pneumonitis. First indications are positive, bringing hope to many more pulmonary fibrosis sufferers. Nintedanib has also recently been shown to be effective against pulmonary fibrosis caused by the autoimmune diseases, scleroderma.

Purple background with white text reading "breaking news"

We are delighted to announce our campaign, backed by patients, families and medical experts to end the restrictions on anti-fibrotic drugs has successfully reached its next step.

Today (28th May 2021) NICE have agreed to review its decision that currently restricts access to anti-fibrotic treatments. Patients currently only receive these treatments when their lung function dips below 80%.

This would not have been possible without the overwhelming support from patients, families and medical experts who have campaigned for over four years to make anti-fibrotic drugs available immediately after a patient  is diagnosed with idiopathic pulmonary fibrosis

“NICE’s decision to re-appraise antifibrotic medicines for IPF is the news our- patient community has been waiting for.  It will hopefully result in all patients having access to these treatments. I know from talking to patients and their families this news offers hope but they are disappointed that the appraisal will take a year to complete. We need to ensure that access to these life-extending medicines happens as quickly as possible”. – Steve Jones, Chair of Trustees

The re-appraisal takes 50 weeks but APF is now urging NICE and Pharma companies to fast-track the process as further delay will impact thousands of lives.

Louise Wright, CEO:

“This is a landmark moment for patients and their loved ones with pulmonary fibrosis. We couldn’t have achieved this without the support of patients, families, MP’s and clinicians who understand the injustice of this rule.  It is now essential that we keep the pressure on and we call on NICE to FastTrack the process.  Patients can’t afford to wait longer.”

Professor Gisli Jenkins of Imperial College and The Royal Brompton Hospital:

I’m delighted with the NICE decision, which raises the prospect of extending treatment with antifibrotic medicines to all people living with the fatal lung disease, IPF. This would lead to better quality of life and longer lives for thousands of patients in the UK

What happens next?

NICE will conduct a review (called a multi-technical appraisal) which normally takes 50 weeks. Patient organisations like APF and ILD clinicians will form an important part of the process.  APF will ensure NICE understands patients views and experiences.

How long will it take before people with IPF can access anti-fibrotics upon diagnosis?

Realistically we are looking at Summer/Autumn 2022.  NICE’s processes take around 50 weeks, after which the NHS has three months to put these changes into practice.

In the full report from NICE, one drug looks much more expensive than the other. Why is that?

In reality both drugs cost roughly the same per patient when you compare how many tablets each patient takes daily.

Why can’t the pharmaceutical companies lower their prices?

Pharmaceutical companies say they need to remain competitive and re-coup their initial investments into the new drugs.

APF will be keeping the pressure on.  We will stay in regular contact with NICE so these changes happen as quickly as possible and update you with more information when it becomes available.

Email us at if you have any further questions.

Together we are stronger.