Throughout 2017, Pulmonary Fibrosis News Today reported daily discoveries, therapeutic developments, and events related to pulmonary fibrosis. Here, we review the top 10 most-read articles of 2017, with a brief description of what made them interesting and relevant to PF patients, family members, and caregivers.
In this retrospective study, researchers reported that the efficacy of Esbriet (pirfenidone) — an approved therapy for idiopathic pulmonary fibrosis (IPF) — diminishes after six months and may even stop working altogether after one year in patients with advanced disease.
The enzyme called urokinase plasminogen activator (uPA) is a potential new target for anti-fibrotic therapeutics, this study reported. The levels of uPA, a protein found in both urine and blood, were higher in blood and lung tissue samples from IPF patients and correlated with worsening lung functions. Decreasing the expression of uPA led to a reduction of interleukin-6 (IL-6), a key cytokine (signalling molecule) linked to IPF and other lung diseases.
Ofev (nintedanib) and Esbriet (pirfenidone) are two therapies widely used in the treatment of PF, but a deeper understanding of how they work within a person is vital to finding better treatments. With this goal in mind, researchers in Germany developed a new model of lab-grown cells derived from patients. With the system, they found both drugs impacted the formation of collagen fibrils, which underlie the formation of fibrotic tissue. Applying the model may help researchers develop new anti-fibrotic therapeutics.
In this study, researchers at North Carolina State University were able to expand the number of a subset of stem cells that carry regenerative properties, and showing anti-fibrosis and anti-inflammatory effects, in IPF animal models. These lung spheroid cells may be a future way of treating patients with fibrotic diseases.
The therapeutic benefits of Esbriet in IPF may work by improving the function of mitochondria, the cells’ energy factories, according to this study. Specifically, researchers found that Esbriet halted fibrosis progression by promoting the selective degradation of damaged mitochondria — a process known as mitophagy — or by stopping the production of damaging molecules known to promote fibrosis, called mitochondria reactive oxygen species.
Ofev improves IPF outcomes by reducing lung function decline and slowing disease progression, according to the results from a Phase 3b clinical trial (NCT01979952). Computed tomography imaging demonstrated for the first time that six months of Ofev treatment could reduce the progression of patients’ lung fibrosis lesions, compared with a placebo.
An investigational inhalant called TD139 was found to be an effective, safe and a well-tolerated treatment for IPF in a Phase 1b/2a clinical trial (NCT02257177). Following the study’s first part, where the therapy’s safety was tested in healthy volunteers, researchers observed that in IPF patients TD139 showed a good absorption profile, especially in alveolar macrophages. These cells contribute to IPF and are one of the investigative treatment’s major targets.
Acute exacerbations of IPF in an 84-year-old man were resolved by treating him with Ofev, without requiring antibiotics or steroids, reported researchers in this case study. No therapy is currently approved for acute exacerbations, but these findings suggest that Ofev might be a potential one.
While DNA damage is commonly found in cells, so are DNA repair mechanisms. Researchers here discovered that a protein called RAGE was capable of fixing the damage inflicted to DNA molecules and, by doing so, prevent scarring (fibrosis) in the lungs of mice in a disease model. These findings suggest fibrosis is reversible and RAGE may be a way of achieving this.
After revisiting several clinical studies, researchers reported that Esbriet can prolong survival in IPF patients. The effects, however, were not observed in all patients — those undergoing a more rapid lung function decline prior to Esbriet use were more likely to benefit from treatment. Researchers also highlighted that people using Esbriet require close monitoring, and that the benefits of early treatment, compared to risks, have yet to be investigated.
Pulmonary Fibrosis News hopes that these developments, and new reports coming your way throughout 2018, will help to educate, better inform, and improve the lives of people living with PF.
We wish all our readers a happy and inspiring 2018.