What’s the Latest Research on Idiopathic Pulmonary Fibrosis?

Idiopathic Pulmonary Fibrosis (IPF) is a rare, difficult-to-treat lung disease affecting around 100,000 patients in the U.S. While two medications have been approved to slow IPF progression, there are currently no approved treatments that can stop or reverse the disease.

We gathered together some of the latest IPF research, which aims to improve how the disease is diagnosed and find new treatments to stop disease progression.

Improved diagnosis for IPF

Idiopathic pulmonary fibrosis can be difficult to diagnose because it can look like other, similar diseases in lung scans. In fact, it can take one to two years for patients to get an accurate diagnosis after symptoms start. Since IPF is a progressive disease, it's important to get the right diagnosis as quickly as possible.

Most cases of IPF are diagnosed by high-resolution computed tomography (HRCT) to identify the hallmark patterns of IPF. This approach can yield inconclusive results, which can lead to misdiagnosis. Sometimes, the only way to receive an accurate diagnosis is through a lung biopsy.

A new diagnosis option aims to make diagnosis clearer through a nonsurgical procedure. The Envisia Genomic Classifier is a genomic test that takes samples through nonsurgical trans bronchial biopsy. The test then detects the genetic pattern in the lungs associated with IPF at a higher accuracy than HRCT does alone, research shows.

The new technology combines gene expression data from bronchoscopy samples with machine learning to identify the patterns associated with IPF. Researchers hope the new approach will improve IPF diagnosis and help patients access the right treatments faster.

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New investigational treatment enters Phase I testing

An early-phase potential new treatment for IPF has also recently entered clinical trials. Indalo Therapeutics launched a Phase 1 clinical trial testing a potential new treatment for a range of fibrotic diseases, including IPF and nonalcoholic steatohepatitis (NASH), an inflammatory liver disease.

Fibrosis is the formation of excessive fibrous connective tissue in an organ. In response to injury, this process is called scarring, but in conditions like IPF, the cause of scarring is unknown. Conditions related to fibrosis have a high unmet medical need, said Bill Bradford, PhD, MD, chief medical officer at Indalo, in a press release.

The therapy aims to block the activation of a process linked to fibrosis. Earlier preclinical studies in animals showed anti fibrotic effects in several organs, including the liver, lung, and kidney.

The Phase 1 trial will test the treatment for safety in healthy volunteers first.

IPF treatment is safe and effective for both early and late stage disease

Later-stage IPF patients were excluded from most clinical trials for Esbriet, one of the few treatments approved for IPF. But, one Phase 3 trial of the drug did not have specific requirements around disease phase. An ad-hoc analysis of that trial has been released, and found that Esbriet is both safe and effective in late stage disease, as well as early-stage patients.

The study analyzed changes in lung function for both early and late-stage patients, and found that both groups had a similar rate of lung function decline, showing Esbriet was effective for both. While there is a need for more effective treatments for all patients with IPF, researchers concluded that Esbriet is a treatment option for later-stage patients.

New treatments for IPF can't move forward without clinical trial participants. Consider taking part in research to help everyone living with IPF.

Featured clinical trial

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