Top 10 Pulmonary Fibrosis Stories of 2019

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by Patricia Inácio, PhD |

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Throughout 2019, Pulmonary Fibrosis News provided you daily coverage of breakthrough discoveries, promising therapies, and clinical trials related to pulmonary fibrosis (PF). As we look forward to bringing more news to patients, family members, and caregivers dealing with PF in 2020, here are the top 10 most-read articles of 2019, with a brief description of what made them interesting to the PF community.

No. 10 – “AstraZeneca Turns to Artificial Intelligence to Speed Discovery of New Medicines for IPF

AstraZeneca announced a collaboration with BenevolentAI to use its expertise in artificial intelligence (AI) and machine learning to aid the development of new medicines for idiopathic pulmonary fibrosis (IPF) and chronic kidney disease (CKD). Benevolent’s AI system has the ability to analyze vast amounts of complex biomedical information, such as data on clinical trials, genes, targets, diseases, proteins, and medicines to propose unbiased new hypotheses and extrapolate previously unknown relationships much more efficiently than human beings can. Benevolent’s AI expertise will be combined with AstraZeneca’s genomics, chemistry, and clinical data expertise.

No. 9 – “Promedior’s Experimental IPF Therapy PRM-151 to Enter Phase 3 Stage

Results from a Phase 2 trial (NCT02550873) in IPF patients showed that a 24-week treatment with Promedior‘s PRM-151 slowed the loss of lung function and significantly enhanced participants’ exercise capacity. Based on those promising findings, Promedior announced the launch of a larger Phase 3 study after receiving a green light on the trial’s design by the U.S. Food and Drug Administration (FDA).

No. 8 – “IPF Patients Live Longer on Ofev Compared with Placebo, Analysis Suggests

Ofev (nintedanib), marketed by Boehringer Ingelheim in the U.S. and as Vargatef in the E.U., was found to significantly prevent disease progression in people with IPF by slowing the rate of decline in forced vital capacity, a measure of lung function. An analysis of pooled data from six clinical trials showed that Ofev also significantly extended the survival of IPF patients compared with a placebo. Those treated with Ofev had an estimated mean survival of 11.6 years, which was approximately three times longer than that predicted for patients treated with a placebo (3.7 years).

No. 7 – “Vitamin D Deficiency Linked to Disease Severity, Mortality Risk in IPF Patients, Study Shows

A study early in 2019 showed that vitamin D deficiency in people with IPF is linked with functional and clinical predictors of disease severity, and a higher risk of mortality. The results also revealed that oral administration of vitamin D partially reversed lung fibrosis, or scarring, in a mouse model. Further, the team suggested that vitamin D levels below a threshold of 17.9 ng/ml identifies IPF patients with a greater likelihood of mortality.

No. 6 – “NYC Rehab Therapist Offers PF Patients Novel Approach to Exercise and Breathing

Our sixth most-read article of 2019 highlighted the work of Noah Greenspan, DPT, a New York pulmonary physical therapist with more than 20 years’ experience training IPF patients in how to breathe better, and how to increase their exercise tolerance. Greenspan runs the Pulmonary Wellness & Rehabilitation Center in midtown Manhattan, and has treated more than 100,000 people with PF, pulmonary hypertension (PH), and chronic obstructive pulmonary disease (COPD) — all lung conditions characterized by shortness of breath.

No. 5 – “Exhaled Nitric Oxide a Potential Biomarker for IPF Severity and Progression, Study Suggests

High levels of nitric oxide in the lung sacs — called alveoli — of IPF patients are linked with faster disease progression and worse survival, according to a study by researchers in Italy that was published in May. More specifically, IPF patients with high nitric oxide levels — six parts per billion (ppb) units or more — had worse outcomes, the findings showed. Meanwhile, those with levels lower than six ppb had an improved survival rate. The study’s findings also showed that a patient’s exhaled nitric oxide level was found to be a strong predictor of IPF progression.

No. 4 – “Data Support Use of Diabetes Medication Metformin to Treat IPF

Metformin, an oral medication for treating type 2 diabetes, was found to suppress the production of collagen, and help in the recovery from lung fibrosis (scarring) in a mouse model of IPF. In a study published in July, researchers showed that metformin suppressed collagen deposits — a key event in lung fibrosis — and induced a switch in lung fibroblasts that is associated with a faster recovery from fibrosis. At the molecular level, the team found that the gene BMP2 showed the most significantly increased expression with metformin treatment, and that it was associated with the inhibition of collagen production.

No. 3 – “PRM-151 Receives FDA Breakthrough Therapy Designation for IPF Treatment

Our third most-read article of 2019 covered the FDA’s decision to grant breakthrough therapy designation to PRM-151, being developed by Promedior for the treatment of IPF. This designation is intended to accelerate the development of potential medications for serious conditions with an unmet medical need. The FDA’s decision was based in part on the success of a Phase 2 clinical trial (NCT02550873) comparing PRM-151 treatment with a placebo in 111 IPF patients. The results showed that those treated with PRM-151 had slower lung function decline and better exercise capacity. PRM-151 is the first compound to receive this designation for IPF since 2014.

No. 2 – “Despite Benefits, Many IPF Patients Switching from Esbriet to Ofev Stop Treatment, Possibly Due to Low BMI, Study Says

Many IPF patients switching from Esbriet (pirfenidone, marketed by Genentech) to Boehringer Ingelheim‘s Ofev (nintedanib) end up discontinuing treatment — especially if they are underweight or have anorexia while being treated with Esbriet, according to a Japanese study. These findings, reported in an April story in Pulmonary Fibrosis News, support the need for careful monitoring of body weight, and vigilance over the nutritional status of patients receiving anti-fibrotic therapies, the researchers noted. According to the team, further investigation is required to establish an optimal treatment strategy.

No. 1 – “Galapagos Launches Global ISABELA Program Testing GLPG1690 in IPF Patients

The most-read PF article of 2019 covered the launch of the worldwide ISABELA Phase 3 program, by Galapagos, to evaluate the potential of its investigational therapy GLPG1690 for treating  idiopathic pulmonary fibrosis. GLPG1690 inhibits autotaxin, an enzyme that is thought to be involved in tissue scarring (fibrosis) and IPF progression. The ISABELA program consists of two identically designed trials, called ISABELA1 (NCT03711162) and ISABELA2 (NCT03733444), being conducted in multiple clinical sites, including in the United States and Europe. The program is expected to enroll 1,500 patients, ages 40 and older, who have received an IPF diagnosis within 5 years of starting the trials. Previous data showed that a daily 600 mg dose of GLPG1690 resulted in an improvement in lung function at 12 weeks in IPF patients. The treatment was well-tolerated, with most side effects being mild-to-moderate, the data showed.


At Pulmonary Fibrosis News, we hope that these articles, along our reporting throughout 2020, help inform and improve the lives of everyone dealing with PF. We wish all our readers a happy 2020.

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