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European Respiratory Society Congress Report 2014






IPF Research Update from the ERS Congress 2014

This year the European Respiratory Society (ERS) Congress took place in Munich, Germany, from 6th to 10th September. 22,000 doctors, nurses, scientists and healthcare workers from all over the world attended the congress, making it one of the largest medical conferences in the world. The ILFA delegates were Denise Dunne and Nicola Cassidy and they were joined by Irene Byrne, Senior Physiotherapist at the Mater Misericordiae University Hospital.

This year the congress programme contained many sessions dedicated to Idiopathic Pulmonary Fibrosis (IPF) including two evening symposia that were packed to capacity with over 300 people in the audience. When there were no seats left, people sat on the floor of the auditorium such was their interest in learning more about the latest research and developments in IPF. One doctor commented on greatness of having a large audience, contrasting it to the audience of 15 people who attended his lecture on IPF at the 1996 ERS Congress in Stockholm.

Another leading respiratory doctor proclaimed that "2014 was the year of IPF"! This excitement reflected the upbeat mood of the audience following the publication of much anticipated results from three clinical trials that were announced in May 2014.

Two of these clinical trials (one with the drug Pirfenidone and one with Nintedanib) were successful and the medications under investigation slowed down the progression of IPF in patients with mild to moderate fibrosis over a 1-year period . A third clinical trial showed that the drug N-acetylcysteine (also called NAC) was ineffective for IPF when used alone as a treatment. During the congress, there were many talks about the finer details of the recent research results for Pirfenidone and Nintedanib, ongoing research studies and future plans.


Pirfenidone is a drug that slows down the rate of lung fibrosis and improves survival for IPF patients with mild to moderate disease. It is only recommended for IPF and cannot be used for treating other types of lung fibrosis. Pirfenidone was approved for use (licensed) by the European Medicines Agency in 2011 and can be prescribed by respiratory consultants for patients who meet strict criteria.

After a drug is licensed, further research studies are carried out to determine the long term safety profile of the medication and to detect any new side effects in patients receiving treatment. Ongoing studies have shown that most patients tolerate Pirfenidone quite well but side effects can occur, as with all medications.

The side effects of Pirfenidone can include stomach upset, rash following sunlight exposure and rarely changes to liver function tests. Side effects usually occur within 6 months of starting the medication but very few patients have had to stop taking Pirfenidone completely . Experience has shown that the best way for doctors to manage the side effects of Pirfenidone is to:

  • educate patients on how to prevent these effects;

  •  to adjust the dose of medication when a side effect develops;

  •  to stop the medication for a short time if necessary;

  •  to re-start the drug when the side effect has resolved.

    These steps help improve the tolerability of Pirfenidone and ensure that patients can continue on the medication and benefit from its actions. Ways to reduce the impact of side effects with Pirfenidone include advising patients to take the tablets with food or at intervals over the course of a meal, and wearing a hat and sun-block while outdoors.

    Preliminary results from an ongoing study called the "RECAP" study have shown that long term treatment with Pirfenidone for up to 5 years is safe and tolerable and has a good survival benefit. An estimate of the survival rate was calculated as 69% for patients taking Pirfenidone for over 4 years.

         It is important to remember that these results are based on the experiences of large numbers of patients around the world rather than looking at individual cases. Furthermore no patient has perfectly stable IPF, even when they are on medication.



Nintedanib has recently been shown to slow down the rate of IPF progression in IPF patients with mild to moderate disease. It works by modifying the activity of growth factors (naturally occurring substances in the body that are necessary for growth and normal function) in the lung. The clinical called INPULSIS 1 and INPULSIS 2 were carried out for 1-year and patients with mild to moderate IPF. Patients on Nintedanib had a lower rate of decline in the lung function tests which is consistent with a slowing down of their lung fibrosis.

The combined results for the INPULIS 1 and 2 clinical trials also revealed a trend towards an improved quality of life and a reduced rate of acute exacerbations (rapid worsening of IPF) in patients on Nintedanib but the results were not statistically significant (a mathematical calculation used to show consistency) because of the low numbers involved.

To validate these results, combinations of results (including the impact of missing results for lung functions tests due to the death of a patient or withdrawal from the study due to illness) and variations for the rate of decline for lung function tests, were analysed using different mathematical calculations. When all of the information was scrutinised using strict research methods, the results were reliable and proved that Nintedanib was an effective treatment for IPF patients with mild to moderate disease.

Nintedanib is being assessed by the European Medicines Agency and may be licensed for treating patients with mild to moderate IPF in the coming months.


Biological and genetic research

There were several talks about the biological and genetic aspects of IPF. IPF is a very complex disease with many cell reactions involved in the disease process. In order for IPF to develop, there must be a genetic susceptibility (weakness or vulnerability) and a trigger (or triggers) to start the process of fibrosis (scarring) in the lungs and cause it to carry on out of control.

There are a number of genes that are believed to be involved in IPF development. While some people with the gene abnormality may be more susceptible to developing IPF, this does not automatically mean they will develop the condition. The triggers thought to be implicated include viruses, bacteria, tobacco smoke or an inhaled chemical or particle but at the moment the exact cause of IPF is not known.

When lung tissue from a healthy adult is examined under the microscope, there are subtle cell changes present that are related to the natural aging process. In order to learn more about the development of IPF and the different pathways involved, experiments need to be adapted to look at "aged lungs" (older lungs) rather than using the "young lung" model that was used in the past.

Studies looking at the presence of different bacteria in the lungs are ongoing to see if there is an increased risk of disease progression associated with specific bacterial strains. Results to date show that there is a wide variety of different bacteria and viruses found in the lungs of IPF patients but the type of bacteria detected varies depending on which part of the lung is looked at and how samples are obtained.

It is hoped that genetic and molecular research will help identify the early processes involved in IPF development and this in turn will help scientist to better understand and predict the response to medications and treatments.


ILFA 2000 Steps a Day Poster Discussion

This year ILFA was selected to discuss our work on the 2000 Steps a Day Exercise Challenge for Lung Fibrosis Patients in an educational session called "Latest insights in physical activity, exercise testing and muscles".

A poster describing the pilot study carried out to assess the usefulness of the ILFA exercise challenge was prepared by Nicola Cassidy from ILFA and Irene Byrne, Senior Physiotherapist from the Mater Misericordiae University Hospital, who jointly developed the novel exercise challenge.  The poster described the development of the exercise programme and how it was successfully trialled and assessed by a group of 15 lung fibrosis volunteers. The feedback from the IPF patients was hugely positive and led to the production of the ILFA 2000 Steps a Day Challenge literature and resources.

Irene Byrne also gave a 5 minute presentation to the audience and answered questions about the ILFA exercise programme. Professor Sally Singh (Head of Pulmonary and Cardiac Rehabilitation and Consultant Clinical Scientist at University Hospital Leicester), who chaired the session, commented that the ILFA 2000 Steps a Day Exercise Challenge was "terrific" and "a really nice programme" and gave us plenty of encouragement.

There was great interest from doctors, nurses, physiotherapists, and researchers about the 2000 Steps exercise programme. ILFA received requests from healthcare professionals from Portugal, Germany, Switzerland and the Czech Republic to learn more with a view to developing a similar programme for lung fibrosis patients in their countries.


And finally ...

The 2014 ERS Congress was a great success and highlighted many advances in IPF research and developments. The coming years promise exciting and dynamic developments to help our understanding of this complex disease.


ILFA's attendance at the 2014 ERS Congress was kindly supported by an educational grant from InterMune.