4 potential pulmonary fibrosis therapies to watch

IPF image from Wikipedia

The drug development landscape in idiopathic pulmonary fibrosis is changing rapidly. “There are probably 30 or 40 different compounds in development,” said Dr. Toby Maher, a presenter at the Pulmonary Fibrosis Foundation (PFF) Summit held last month. Of those, a handful have reached Phase 2 and Phase 3 clinical trials. Here, we highlight a handful of pulmonary fibrosis therapy candidates.

1. Pamrevlumab

One of the pathways drug developers have explored to treat IPF involves connective tissue growth factor (CTGF). The protein signals to cells to stimulate the development of fibrotic tissue In IPF. “We know that there’s an excess of CTGF,” Maher said. “And we know that that can be blocked using antibodies.”

This observation led to the development of a monoclonal antibody first called FG3019 and later rechristened as pamrevlumab.

Developed by FibroGen (NSDQ:FGEN), Pamrevlu…

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Why Aria Pharmaceuticals is upbeat about two novel IPF treatment candidates 

Small molecule drug developer Aria Pharmaceuticals (Palo Alto, Calif.) recently revealed that two investigational treatments for idiopathic pulmonary fibrosis (IPF), TXR-1002 and TXR-1007, demonstrated promising results in preclinical research.

There are currently only two FDA-approved treatments for IPF on the market — nintedanib (Ofev) from Boehringer Ingelheim and pirfenidone (Esbriet) from Roche. While those introductions marked a turning point in treating IPF, managing the disease remains challenging for many patients.

Both TXR-1002 and TXR-1007 rely on a mechanism of action than that of nintedanib or pirfenidone. And Aria reports that preclinical testing indicated that the drugs were well tolerated. “Certainly, you can’t tell if a rat is suffering from nausea or something like that, but at a gross level, the animals did well through the course of the study,” said Mark Eller, senior vice president, R&D at Aria Pharmaceuticals. “We’re optimistic based o…

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Why pulmonary fibrosis deserves more attention

Idiopathic pulmonary fibrosis x-ray courtesy of Wikimedia Commons.

This September is the inaugural Pulmonary Fibrosis Awareness Month, which the Pulmonary Fibrosis Foundation and allies have launched to educate the public about the disease family. Involving scarring of the lungs, pulmonary fibrosis gradually robs the breath from patients it affects. 

Some 200,000 Americans live with idiopathic pulmonary fibrosis.  

At present, two drugs are available to treat the condition in the U.S., but pulmonary fibrosis remains an active focus area for drug developers.

Given that September is Pulmonary Fibrosis Awareness Month, the anniversary of the September 11 attacks also can serve as a reminder of the impact of interstitial lung disease. “Lots of lung disease has occurred in the first responders of the World Trade Center,” said Dr. Joyce Lee, a senior medical advisor for the Pulmonary Fibrosis Foundat…

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The pulmonary fibrosis treatment landscape: An interview with an expert

Photo by Anna Shvets from Pexels

In 2014, FDA approved two medications for idiopathic pulmonary fibrosis (IPF) — nintedanib from Boehringer Ingelheim and pirfenidone from Roche (OTCMKTS:RHHBY). “That was a huge success for our community but certainly, it’s the beginning of what we need to be doing for our patients,” said Dr. Joyce Lee, a senior medical advisor for the Pulmonary Fibrosis Foundation, a nonprofit dedicated to supporting research and medical advances related to the treatment of the condition.

Idiopathic pulmonary fibrosis, which is associated with shortness of breath and a significant symptom burden, has no known cause or cure.

[Related: 6 notable pulmonary fibrosis research efforts]

But there is reason for hope, according to Lee, who is also a professor of medicine specializing in pulmonary sciences and critical care at the University of Colorado. “There is a lot of investigation …

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6 notable pulmonary fibrosis research efforts 

Image courtesy of Pixabay

Idiopathic pulmonary fibrosis (IPF) is frequently a debilitating disease associated with significant morbidity and mortality. Although a rare disease, its incidence has increased in recent decades, and it leads to more deaths than some cancers. 

The treatment landscape for the condition changed in 2014 when FDA approved the first drugs indicated for IPF, nintedanib from Boehringer Ingelheim and pirfenidone from Roche (OTCMKTS:RHHBY). 

[Related: The pulmonary fibrosis treatment landscape: An expert interview]

Research on pulmonary fibrosis has increased in recent years. 

Here’s a summary of notable IPF research: 

Boehringer Ingelheim has an international study investigating a phosphodiesterase 4b (PDE4b) inhibitor in patients with idiopathic pulmonary fibrosis. Researchers have found that the drug has anti-fibrotic effects in animal models and lung fibrosis …
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