Ferrer obtains the distribution rights of a potential treatment for Idiopathic Pulmonary Fibrosis (IPF) and Progressive Pulmonary Fibrosis (PPF)
Barcelona, May 27h, 2024. The international pharmaceutical BCorp company Ferrer continues to strengthen its focus on pulmonary vascular and interstitial lung diseases by announcing the expansion of its current distribution agreement with United Therapeutics Corporation (Nasdaq: UTHR) for Tyvaso® (treprostinil inhalation solution*) – a pharmaceutical drug-device combination product authorized for the treatment of WHO Group 3 Pulmonary Hypertension associated with Interstitial Lung Disease (PH-ILD), and for WHO Group 1 Pulmonary Arterial Hypertension (PAH).
Both companies have signed the distribution rights for two new potential indications of the molecule, Idiopathic Pulmonary Fibrosis (IPF) and Progressive Pulmonary Fibrosis (PPF), two rare diseases characterized by scarring of lungs which results in difficulty for patients to breathe, leading to poor quality of life and even death. Current available treatments for IPF and PPF have only been shown to slow disease progression, whereas treprostinil inhalation solution could potentially offer a new treatment option in these patients.
“The pulmonary vascular and interstitial lung diseases field is very important for us”, said Óscar Pérez, Chief Scientific and Business Development Officer at Ferrer. “Strengthening our partnership with United Therapeutics Corporation can advance our purpose of using business to fight for social justice through the delivery of new solutions for people, families and caregivers living with devastating diseases”.
Under the expanded agreement, Ferrer has been appointed as the exclusive, worldwide distributor (except for the US, Canada, Japan, China, and Israel), to make this therapeutic option available upon approval for the treatment of IPF and PPF and keep delivering transformative solutions to the patients in need.
The announcement leverages Ferrer’s strong capabilities and expertise in pulmonary vascular and interstitial lung diseases and underscores both companies’ commitment to expanding global access to treprostinil inhalation solution for IPF and PPF patients, if approved.
Treprostinil inhalation solution is currently being assessed in three Phase 3 clinical trials: two in IPF patients (with over 1.000 subjects combined), and one in PPF patients (with 698 subjects), with a 52-week follow-up and collectively covering a global study geography.
* Tyvaso® (treprostinil inhalation solution is a prostacyclin mimetic for PH-ILD is currently authorized in the US, Israel, Chile, Dominican Republic, and Argentina, and under regulatory review in Japan, and for WHO Group 1 Pulmonary Arterial Hypertension (PAH), currently authorized in the US, Israel, Argentina, Colombia, Japan, and Dominican Republic. Regarding the registration of Tyvaso® (treprostinil) inhalation solution for the treatment of PH-ILD, Ferrer has just obtained the marketing authorization in Chile and Dominican Republic and will focus on obtaining it in the United Kingdom as well. Further assessment of Tyvaso® PH-ILD for the European Union will continue.
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About Interstitial lung disease (ILD)
Interstitial lung disease (ILD) is a group of lung diseases that are characterized by marked scarring or fibrosis of the bronchioles and alveolar sacs within the lungs.
About Idiopathic pulmonary fibrosis (IPF)
Idiopathic pulmonary fibrosis (IPF) is a scarring disease of the lungs of an unknown (idiopathic) cause and is the most common of the idiopathic interstitial pneumonias. IPF is characterized by the progressive loss of the ability of the lungs to transfer oxygen into the blood, the condition can present with a wide range of symptoms, including shortness of breath with activity, labored breathing, and fatigue, ultimately resulting in respiratory failure and death. While the precise causes of IPF remain unknown, IPF rarely presents before age 50 and can be associated with cigarette smoking and certain genetic dispositions. In addition, some evidence suggests that gastroesophageal reflux (acid reflux, or heartburn), certain viral infections, air pollution, and some exposures in the workplace may be risk factors for IPF. According to recent research, IPF is estimated to affect between 0.33 and 4.51 people per 10,000 persons worldwide, and prognosis is poor, with a median survival of 2–3 years if left untreated1.
About Progressive pulmonary fibrosis (PPF)
Progressive pulmonary fibrosis (PPF) is a group of interstitial lung disease (ILD) conditions that exhibit progressive, self-sustaining fibrosis, and display a similar disease course to idiopathic pulmonary fibrosis (IPF). PPF includes idiopathic interstitial pneumonias (other than IPF), autoimmune ILDs, chronic fibrosing hypersensitivity pneumonitis, and fibrotic ILDs related to environmental or occupational exposure. It is estimated that 13% to 40% of patients with these various ILDs will go on to develop PPF2. Patients with PPF exhibit decreased lung function, poor quality of life, and increased mortality despite usual treatments for the underlying ILD. Estimates for median transplant free survival and overall survival are approximately 2.9 years and 3.7 years, respectively3,4.
About Pulmonary hypertension associated with Interstitial lung disease (PH-ILD)
Pulmonary Hypertension (PH) frequently complicates the course of patients with interstitial lung disease and is associated with worse functional status measured by exercise capacity, greater supplemental oxygen needs, decreased quality of life, and worse outcomes. PH is estimated to affect at least 15% of patients with early-stage ILD and may affect up to 86% of patients with more severe ILD5.
References
1 Maher TM, Bendstrup E, Dron L, et al. Global incidence and prevalence of idiopathic pulmonary fibrosis. Respir Res. 2021 Jul 7;22(1):197.
2 Olson AL, Patnaik P, Hartmann N, et al. Prevalence and incidence of chronic fibrosing interstitial lung diseases with a progressive phenotype in the United States estimated in a large claims database analysis. Adv Ther. 2021;38:4100-4114.
3 Platenburg MGJP, van der Vis JJ, Grutters JC, van Moorsel CHM. Decreased survival and lung function in progressive pulmonary fibrosis. Medicina. 2023;59:296.
4 Cottin V, Teague R, Nicholson L, et al. The burden of progressive-fibrosing interstitial lung disease. Front Med. 2022;9:799912.
5 Behr J, Nathan SD. Pulmonary hypertension in interstitial lung disease: screening, diagnosis and treatment. Curr Opin Pulm Med. 2021 Sep 1;27(5):396-404.