Fitness for breath 2018: a bike tour in the center of Italy and the first Italian meeting on IPF and other rare respiratory diseases

FIMARP, the Italian Federation of support groups for patients with IPF is organizing the first Italian meeting on IPF and other rare respiratory diseases in Pistoia from September 22-23. This meeting will be an important occasion to bring together doctors, patients and all the relevant stakeholders involved in rare pulmonary diseases. It will be followed by a triangle tournament of football between Italian Transplanted Team, Italian National Transplanted Team and an all stars Italian team and a concert (Music 4 Breath) in memory of the first FIMARP President Alessandro Giordani.

Other initiatives will take place in some Italian cities during IPF World Week 2018, including a spirometry day in Catania and in Padua.  

Additionally, as part of the Fitness 4 Breath initiative, a bike tour started on August 26: Achille Abbondanza, a 46 years old IPF patient will bike from Rome to Forli' (525km) in 1 week to raise awareness of IPF. Other IPF patients can also join his tour and he will stop in different cities to visit the local hospitals and the patients support groups.  

Additional information on this event is available on FIMARP website: 


IPF World Week in Italy: Yoga and breathing exercises

During IPF World Week 2018, the Italian support group for patients with IPF AMA FUORI DAL BUIO will organize a yoga session focusing on breathing together with AnatomYoga® . The session will take place on September 23 from 10:00am to 1:00pm in Modena (Italy). 

Yoga is beneficial for IPF patients as it can stimulate the lungs through focused breathing. In fact, basic breathing exercises may improve lung function and ease breathing problems.

Additional information on this event is available on the following websites - - and at the following email address:


IPF Week in Poland

The IPF Polish Society is organizing an event on September 22, 23, 2018. On Saturday, September 22 three lectures about IPF will be given by healthcare professionals specialised in IPF, who will be also meeting with patients with IPF. On Sunday, September 23 a picnic for doctors, nurses, patients and their families will be organized; this will also be an opportunity to have spirometries performed. One of the objectives of this event is to continue collecting money for portable oxygen concentrations. 

The programme of the event is available at this link and additional information is available on the website of the IPF PolishSociety


IPF World Week in Italy: Open Day with healthcare professionals and patients

During the IPF World Week 2018, the Italian support group for patients with IPF AMA FUORI DAL BUIO will organize an Open Day with healthcare professionals and IPF patients at the Center for Rare Pulmonary Diseases at the Modena's hospital (Largo del Pozzo, 71 from 10:00am to 1:00pm). The aim of this event is to provide patients and their carers with up to date information on the latest research developments in IPF. Additionally, the event is supposed to be not only a moment during which patients can share their own experiences and best practices with other patients living with IPF, but also an occasion for researchers and doctors to present the new developments in the field of diagnosis and IPF care. The event will see the participation of Dr. Luppi, the director of the Center for Rare Pulmonary Diseases, Dr. Cerri, researcher at the same Center and Dr Magnani, coordinator of the IPF support group, together with Rosalba Mele, president of AMA FUORI DAL BUIO.  In the framework of this event, it will also be possible to benefit from free spirometries from 10:30am to 12:30pm. 

Additional information on this event is available on the following websites - and at the following email address:


Talk about it! 10 meetings in 10 hospitals.

The Dutch Pulmonary Fibrosis Society in cooperation with 10 hospitals is organising 10 meetings in 10 different hospitals in the Netherlands during the IPF World Week 2018. During these meetings, patients, carers and healthcare professionals will learn more about pulmonary fibrosis. Different topics will be covered, among which: diagnosis, treatment, oxygen therapy and the impact of the disease in patients' daily lives.  

The Dutch Pulmonary Fibrosis Society will also take this opportunity to present its activities and to launch its new campaign: 'You don't have pulmonary fibrosis by yourself, talk about it!'.  The aim of this campaign is to encourage patients living with pulmonary fibrosis to seek the support of their carers and to educate the carers on how to support their loved ones. 

Additional information is available at this link and at the following email address:



Shifting paradigms in the diagnosis and treatment of ILDs

Since the last meeting in 2016, there have been many exciting advances in the field of interstitial lung diseases (ILDs). The Ruhrlandklinik Essen hospital will present an extensive update on clinical and research developments in the field of idiopathic pulmonary fibrosis (IPF) and other ILDs. The 6th ILDs Colloquium will take place on 21 September 2018 at the Ruhrlandklinik (Essen, Germany), part of the University of Medicine of Essen. Francesco Bonella, chairman of the EU-IPFF Scientific Advisory Board is featured among the speakers. The full programme is available here

Additional information on this event is available at this link and at the following email address:




International Symposium: Diagnosis and therapies in diseases with telomere shortening: dyskeratosis congenita and pulmonary fibrosis

Madrid, 20-21 September 2018

The Madrid headquarters of the Ramón Areces Foundation will host the 'International symposium on diagnosis and therapies in diseases with telomere shortening: dyskeratosis congenita and pulmonary fibrosis’. This meeting of experts, coordinated by María Molina, member of the EU-IPFF Scientific Advisory Board, among other coordinators, will place special emphasis on two of these rare diseases, dyskeratosis congenita and pulmonary fibrosis.

In addition, Carlos Lines, President of the Spanish IPF Association (AFEFPI) and of the European IPF Federation (EU-IPFF),  will highlight the importance of the associative movement to fight against the impact of these pathologies from a Spanish and a European perspectives.

The full programme is available at this link

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EU-IPFF publishes an article on Health Europa Quarterly

Carlos Lines Millán, the President of the European Idiopathic Pulmonary Fibrosis and Related Disorders Federation (EU-IPFF), was interviewed by Health Europa, on “The challenges of idiopathic pulmonary fibrosis”.

This was a great opportunity to raise awareness on this unknown disease, and to promote the Federation’s activities and goals, including timely diagnosis and an equal standard of care across Europe.

Read the full article here:



To access the full version of the article, please visit:

Article originally published on:
Reproduced by kind permission of Pan European Networks Ltd,

© Pan European Networks 2018



Highlights of the ATS congress 2018- By Francesco Bonella

Francesco Bonella, chairman of the EU-IPFF Scientific Advisory Board, participated in the American Thoracic Society Conference (ATS) in San Diego from 18-23 May 2018. Find below a short overview of the most recent advances in the research and care of IPF, as highlighted at the conference.


New data on pathogenesis and biology of IPF

The issue of immunological pathways in IPF seems to be attracting more attention than ever from researchers. The activation of lymphocytes through the IL17 pathway (Th17 lymphocytes play also a role in autoimmune diseases and asthma) and overexpression of PD1 (an immune checkpoint protein which can also prevent the immune system from killing cancer cells), on macrophages, fibroblasts and lymphocytes, which in turn trigger TGF-β fibrotic pathway (Geng Y, et al. ATS San Diego 2018; C73: PA5782), could open the way to new treatments in IPF.

Preservation of mitochondrial DNA is essential for the survival of cells. Its instability as a consequence of oxidative stress seems to be associated with activation of profibrotic pathways and defective cellular death (Kim S. ATS San Diego 2018; C85). Mitochondria directed therapeutics could therefore become an option for lung fibrosis.

New clinical trials in IPF:


The RIFF study was a phase II study on the efficacy of humanized monoclonal antibody binding IL-13, lebrikizumab, in IPF. It showed no benefit on lung function or mortality of 250 mg subcutaneous lebrikizumab given every month over 52 weeks in patients with IPF. Results suggest that targeting the IL-13 pathway alone may not be sufficient for achieving a therapeutic benefit in patients with IPF (Swigris JJ, et al. ATS San Diego 2018; D12: OA6167).

The ESTAIR Study was a phase II trial to test monoclonal antibody binding to IL-13 and IL-4, SAR156597, in IPF. The study found no difference between treatment and placebo group in lung function, death or all-cause mortality. The safety profile of SAR156597 was also unfavorable (Raghu G, et al. ATS San Diego 2018; A93: OA2441).

For many years, the role of gastroesophageal reflux in the pathogenesis of IPF and as a trigger of acute exacerbations has been under debate. The phase II trial “weighing risks and benefits of laparoscopic anti-reflux surgery in patients with IPF” (WRAP-IPF) did not show any benefit of this procedure on changes in FVC (Forced vital capacity) over 48 weeks. However, treatment was associated with fewer acute exacerbations and deaths, as well as fewer patients with 10% FVC decline. Further investigation is needed to confirm these results (Raghu G, et al. ATS San Diego 2018; A93: OA7698).

…and positive results

Several phase 2 trials with promising preliminary results are ongoing.

A randomized, placebo-controlled, double blind phase II clinical trial to assess primary outcomes (safety and tolerability), pharmacokinetics, and pharmacodynamics of autotaxin inhibitor GLPG1690 (n=15) vs placebo (n=5) over 12 weeks in patients with IPF showed that adverse events were comparable in the treatment and placebo arm. Although the study was not designed or powered to investigate efficacy, FVC and functional respiratory imaging results provided promising preliminary efficacy signals (Maher TM, et al. ATS San Diego 2018 and Lancet Respir Med 2018 May 18. Epub ahead of print)

The results of the randomized, double-blind, placebo-controlled, multicenter trial to test the effect of 28 weeks of recombinant human pentraxin-2 (n=77) on change from baseline in mean FVC % predicted (primary endpoint) vs placebo (n=39) in patients with IPF show a significant effect of recombinant human pentraxin-2 (PRM 151) vs placebo on the mean change in FVC percent predicted over time. Moreover, patients in the treatment arm experienced stabilization in the 6-minute walk test over time, whereas patients in the placebo arm experienced a decline. Adverse events were similar between treatment groups (Raghu G, San Diego 2018 and JAMA 2018 May 20. Epub ahead of print).

A lot of new data from long-term observational studies, as well as real-life experiences with the two approved drugs pirfenidone and nintedanib, confirmed the results on safety and efficacy signals which were already known from the clinical trials. Moreover, promising new data is emerging from quality of life investigations in patients under antifibrotic treatment (benefit on 6-minute walking test and symptoms burden).

The new guidelines on IPF diagnosis were also shortly presented by Raghu Ganesh in an oral session presentation; however, they are still under revision.

In short, the conference was one of the most exciting ones since 2014 – the year in which the results of the phase III trials were published. Just as the 2014 conference led to the approval of pirfenidone and nintedanib as treatment for IPF, the ATS Conference 2018 also gives us new hope for innovative treatments for IPF in the future.

New article on the importance of early diagnosis and treatment in idiopathic pulmonary fibrosis

A new call to improve IPF diagnosis has been launched by some Spanish experts in May 2018.

With an article that recalls the challenges of an early and correct diagnosis of this rare condition, the authors show the benefits of a rapid provision of treatment on the prognosis and quality of life for IPF patients. The authors conclude with recommendations to improve the current state of play: enhanced care management, better coordination among stakeholders, and increasing funding for IPF research and management.

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Challenges in the diagnosis of IPF

IPF shares a challenge with many rare diseases, as its symptoms are often confused with other general respiratory conditions, and this can lead to a significant amount of patients being initially misdiagnosed with asthma, chronic obstructive pulmonary disease (COPD), or pneumonia, among other diseases. In this regard, detection of velcro-like crackles during chest auscultation, which are strongly associated with the presence of lung fibrosis, has been proposed as a feasible and sensitive measure to improve early detection. However, most patients who refer the first symptoms at the primary care physician are symptomatically treated, and only when no improvement is warned, are referred to the respiratory physician.

Diagnosis of IPF requires the presence of a pattern of usual interstitial pneumonia (UIP) on high-resolution computed tomography (HRCT), or combinations of UIP patterns on HRCT and on tissue samples from lung biopsies. Some hope to improve early diagnosis is also given by several clinical trials that are trying to identify molecular markers that are associated with the manifestation of IPF.

Benefits of early diagnosis and treatment of IPF

An early intervention is crucial to improve IPF prognosis. The ATS/ERS/JRS/ALAT guideline for the treatment of IPF includes recommendations for the use of nintedanib, pirfenidone, and antacid therapy, as well as of non-pharmacological treatments such as pulmonary rehabilitation, long-term oxygen therapy, and lung transplantation. Importantly, real world data from a multi-center, prospective, observational registry support the beneficial effect of anti-fibrotic therapy on survival (Hazard Ratio (HR) 0.56, 95%CI 0.34–0.92, p = 0.022) and transplant-free survival independent of baseline disease severity, compared to non-anti-fibrotic therapy.

Need for improved management, better coordination among stakeholders, and increasing funding

A rapid and accurate diagnosis of IPF requires a multidisciplinary discussion among physicians specialized in interstitial lung diseases (ILDs); however,  multidisciplinary teams (MDTs) are only available in tertiary care reference centers. Thus, prompt referral from primary care or non-specialist pulmonology services to centers of expertise is essential for early diagnosis. Lack of quick referral or coordination among centers hinders rapid and accurate treatment and is detrimental to the patient. For instance, an accurate early diagnosis could prevent the misdiagnosis with COPD and treatment with corticosteroids, discouraged in IPF patients.

Despite the widely accepted benefits of referral to a reference center, some respiratory physicians still do not seek support from MDTs for early diagnosis and treatment. In addition, a stronger information flow and support among physicians and patients would be desirable, to manage expectations and avoid misinformation.  An integral multidisciplinary approach in IPF patient will eventually help improve quality of life and treatment adherence.

All these management measures translate into more funding requirements. Since IPF is a rare disease, National Health Systems (NHS) should play a main role in the funding of disease management strategies. Without Health Authorities participation, improvement of management is compromised.