Elena Gobartt, Manager of Specialty Care Medical Affairs at Boehringer Ingelheim Spain.
One in five patients with interstitial lung disease can develop a progressive pulmonary fibrosis (PPF), which affects the patient’s quality of life and is associated with high mortality. Several experts have spoken regarding this during the symposium “Clinical advances in pulmonary fibrosis” that has been held at the 55th Congress of the Spanish Society of Pulmonology and Thoracic Surgery (Separ)from June 2 to 4 in Pamplona, with the aim of continuing to advance in the early diagnostic and the best approach to this pathology.
Las diffuse interstitial lung diseases (DILD) are a diverse group of more than 200 heterogeneous lung diseases, among which idiopathic pulmonary fibrosis (IPF) is the most frequent. When a person has IPF, a rare and progressive disease, the lungs become scarred and stiff, making it difficult for oxygen to get into the blood and causing shortness of breath. IPF is the only ILD that is characterized by always manifesting IPF, but there are other types of ILD that can also manifest it in some cases, thus presenting a similar course and prognosis to those of IPF.
The moderator of the symposium, claudia valenzuela, pulmonologist, head of the ILD Unit of the Pneumology Service at the Hospital Universitario de la Princesa (Madrid) and coordinator of the ILD Area of Separ, explains that: “Progressive pulmonary fibrosis is not an entity in itself, but a behavior that it is present in IPF -the prototype of progressive fibrosing lung disease- since its diagnosis and that it can also occur in other fibrotic ILD. Namely, PPF can appear in other ILDs that are not IPF and that, at some point in the course of the disease, they may present this progressive fibrosing behavior.
“In 2018, a group of experts described this behavior, in certain ILDs, with characteristics similar to IPF, and the first prevalence studies in different cohorts were published. Last May, some upgrade guides in IPF. These include a section that, with all the evidence available to date, has as objective to unify the terminology and talk regarding progressive pulmonary fibrosis”, he added. As the specialist points out, these guidelines “mark the clinical, functional and radiological criteria to be able to define this behavior when it occurs in ILDs that are not IPF”.
PPF is characterized by diffuse, sustained and irreversible fibrosis that worsens respiratory symptoms and reduces lung function, thus slowing the progression of the disease is essential for quality of life and the patient’s prognosis, since it is associated with high mortality. In addition to the functional impact, patients with PPF may experience emotional disturbances, such as depression.
During the symposium, Athol Wellsa specialist in diffuse lung diseases at the Royal Brompton Hospital (United Kingdom), reviewed how the concept of PPF arose and reviewed the evidence that has led to its current definition together with the Mary MolinaHead of the Pneumology Research Group at the Bellvitge University Hospital (Barcelona) and coordinator of the Pulmonary Interstitium Functional Unit (UFIP), who reviewed the recently published update of the ATS/ERS/JRS/ALAT international guide.
Boehringer opens the door to more possibilities
For its part, Luca Richeldi, director of the Division of Pulmonary Medicine at the Gemelli University Hospital (Italy), spoke regarding the safety and efficacy of the drug BI1015550 in the latest Boehringer Ingelheim clinical trial in patients with IPF, since he was the principal investigator of the study. This molecule is an oral phosphodiesterase 4B (PDE4B) inhibitor that, currently under study, might have potential to treat both pulmonary fibrosis and associated inflammation to progressive interstitial fibrosing lung diseases.
Data from the phase II clinical trial obtained at 12 weeks showed a reduction in the rate of lung function decline in patients with IPF, both in those not receiving antifibrotic treatment and in those receiving it. Following these findings, Boehringer Ingelheim will initiate a phase III clinical trial program with the ambition to make this medicine available to patients as soon as possible.
In February 2022, the US Food and Drug Administration (FDA) granted the designation of innovative therapy to this new investigational drug, which is currently not approved in any country for any indication.
Separ young talent research grants
During the congress, Boehringer Ingelheim awarded three research grants for projects presented by members of the Emerging Group of ILD (Geepid), created within the ILD Area of Separ in order to encourage research among its younger members. The objective of these aids, 5.000 euros each one, is to support the research projects that the scholarship recipients have presented in relation to diffuse interstitial lung diseases.
The selected projects have been Candela Serrapulmonologist at the Hospital de la Santa Creu i Sant Pau (Barcelona), on “Characterization of cases of pulmonary toxicity secondary to immunotherapy”; Oswaldo Antonio Caguana Velez, pulmonologist at Hospital del Mar (Barcelona), on “Impact of oxygen therapy on exertion on the activity of patients with ILD who desaturate on exertion”; and the one of Elisa Martínez Besteiropulmonologist at Hospital de La Princesa (Madrid), on “Pleuroparenchymal fibroelastosis: epidemiology, diagnostic characterization, prognosis and management”.
Elena Gobarttmanager of Medical Affairs Specialty Care at Boehringer Ingelheim Spain, points out the company’s commitment to lung diseases that are difficult to diagnose, noting that: “At Boehringer Ingelheim we have a long trajectory in pulmonary fibrosis research, and with this investigational new drug, which has been shown to slow the decline of lung function in patients with IPF, we have the ambition to continue adding value through innovation.
“In line with our commitment to diffuse interstitial lung diseases and independent research, we have awarded three scholarships to members of the emerging Geepid group at the Separ congress, thus sharing with the highest-level specialists in Pulmonology and young talents the need of keep discovering solutions for these diseases. If we all join forces, we will achieve more progress for the future of pulmonary fibrosis”, she continued.
Boehringer Ingelheim has been working hand in hand with scientific societies such as Separ and patient associations for years to promote awareness campaigns that improve knowledge of pulmonary fibrosis; promote valuable programs to help patients and foster cross-training of key specialists in the approach to pulmonary fibrosis. In fact, the Advances in Pulmonary Fibrosis website provides scientific updates, guides and courses to help identify and manage pulmonary fibrosis early in multiple pathologies, including progressive pulmonary fibrosis.
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