Marlies Wijsenbeek and Francesco Bonella, members of the EU-IPFF Scientific Advisory Board participated in the American Thoracic Society Conference (ATS) which took place in Washington from 19-24 May 2017. With around 17,000 attendees, the ATS conference is one of the largest conferences offering ground-breaking research in pulmonary, critical care, and sleep medicine. This year’s congress provided an excellent opportunity to discuss different aspects and up to date research in IPF treatment and care:
Factors influencing IPF onset anddisease course
A study onIPF patients monitored at home revealed that exposure to air pollution could increase the risk of acute exacerbations and mortality (Johannson K.A.). Another study assessing the effect of occupational exposure to metal, wood and agricultural dust on IPF found that the occupational exposure was associated with IPF disease burden. Prevention measures in the workplace could potentially reduce thedisease burden by as much as 13% (Cummings K.J et al).
In addition, Toby Maher from Royal Brompton Hospital reported that the bacterial composition in IPF patients lungs affect the hosts’ immune response and can significantly influence progression-free survival. Also fungicould be involved in the worsening of IPF; however, these new findings require further investigation.
Use of supplemental oxygen during routine daily activities
Although oxygen is often provided for patients with fibrotic interstitial lung disease (fILDs), little research on its benefit exists. A group of UK researchers investigated the impact of supplemental oxygen on routine daily activities and quality of life for people with fILDs. Using patient reported outcome measures, the study showed a significant improvement in breathlessness and activity with the use of ambulatory oxygen. However, no improvement in the psychological domain was found.
Views on IPF management
A study on patients’ and physicians’ views on IPF management (Mahler) found that only 56% of interviewed patients feel that they received enough information at diagnosis, while less than half discussed disease prognosis and were told about treatment options. Most patients felt that the ability of anti-fibrotic drugs to slow disease progression was more important than side effects. Among the patients who had received antifibrotic therapy, 86% felt confident in managing those side effects.
Meanwhile, physicians with more of a watch-and-wait attitude (initiation of anti-fibrotic treatment more than 4 months after diagnosis) were less likely to discuss prognosis and were more concerned about side effects. On the other side, proactive physicians (initiation of anti-fibrotic treatment less than 4 months after diagnosis) were more concerned about disease progression and more likely to discuss prognosis.
Role of the multidisciplinary team in diagnosis and treatment
When it came to diagnostic tools for IPF and ILDs, the focus was on the role of the multidisciplinary discussion (MDD) to reach a definitive diagnosis and to decide the appropriate treatment for patients. A study conducted by Walsh et al came to the conclusion that ILD experts diagnosed IPF correctly more often than non-experts, thereby confirming the diagnostic value of the MDD as a valid tool, especially within reference centres. Another study found that the involvement of a rheumatologist in the MDD led to a diagnosis of connective-tissue disease-related ILD in about 20% of IPF patients, thereby further increasing diagnostic accuracy (Israeli-Shani L. et al).
Current and future treatment options for IPF
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 from the clinical trials. Interestingly, treatment with pirfenidone or nintedanib has not found to be associated with cardiac, bleeding or airway complications in IPF patients who received lung transplantation (Dorey-Stein et al). Moreover, the results of a phase IV single-arm, open-label study on the combined use of pirfenidone and nintedanib in IPF did not suggest a different safety profile to that expected for either treatment alone (Flaherty et al). It should be stressed that for the moment combination treatment can only be used in the context of controlled trials.
Several phase 2 trials with promising preliminary results are ongoing. Prometic‘s PBI-4050 is an orally active lead drug candidate which demonstrated a strong safety profile and promising efficacy, either alone or in combination with nintedanib or pirfenidone.
TD139, an inhibitor of galectin-3 that affects receptor signalling including the pro-fibrotic molecule TGF-β – has been tested against a placebo in 24 IPF patients for two weeks. The compound appeared to be safe and well tolerated and the results regarding efficacy in terms of suppressed Gal-3 expression on BAL macrophages were promising.
In conclusion, the ATS conference 2017 provided new insights in the pathogenesis, management and treatment of IPF and ILD in general, confirming that research and clinical practice are close each other in this rare disease group.