University of Calgary Creating Survey to Help Pinpoint Risks of PF

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by Mary Chapman |

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The University of Calgary is developing a clinical tool to help identify the environmental and occupational risks of pulmonary fibrosis (PF), an undertaking that could lead to a deeper understanding of how the disease takes hold and aid in developing treatments.

During the 18-month project, supported by the Three Lakes Foundation, a comprehensive questionnaire is being designed to pinpoint exposures that could lead to PF risk.

No available treatments can prevent PF progression, and disease screening and diagnostic tools are limited. And despite the importance of inhaled exposures to the risk of interstitial lung disease (ILD) — an umbrella term for diseases that cause scarring (fibrosis) of the lungs, including pulmonary fibrosis — there is no standard approach to characterize such exposures.

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“We know that certain inhaled exposures such as smoking or asbestos can lead to some form of ILDs,” Bridget Burke, associate director of Three Lakes Foundation, said in a press release. “There are other environmental and occupational exposures that could trigger the development of disease, but we don’t have a clear understanding of what those are yet or a standardized tool to help clinicians screen at-risk patients.

“This project will help us to learn more about PF and turn those learnings into action within the clinic setting,” Burke said.

Planned to consist of four stages, the work will be led by Kerri Johannson, MD, director of clinical research for the interstitial lung disease program at the university, located in Alberta, Canada.

“Understanding the exposures associated with PF will help us to discover how the disease evolves and progresses,” Johannson said. “It may also lead us to develop effective interventions and treatments.”

The project opens with a systematic review of medical literature in order to identify all known occupational and environmental exposures that are linked to fibrotic lung disease.

Because there is no single, standardized way to gauge environmental exposures in patients, investigators in the project’s second stage will gather and combine all published ILD questionnaires used by clinicians and research centers.

In stage three, researchers will collaborate with ILD experts from around the world, including those in the U.S., Australia, and the U.K., to establish a consensus and develop a single comprehensive questionnaire to identify specific environmental or occupational exposures that ultimately could lead to a higher PF risk.

The final stage is a pilot test, with the team bringing the questionnaire to people in small prospective registries to judge its performance and ease of use compared with existing tools. Once a final questionnaire is created, it will be made available across the PF community.

Long-term plans include evaluating the questionnaire’s usefulness in large groups of ILD patients, distributing the questionnaire widely to pinpoint other potential exposures, and modifying the questionnaire as new information becomes available.

The Three Lakes Foundation is a nonprofit focused on transforming how pulmonary fibrosis is diagnosed and treated by bringing together in these efforts industry and academic researchers and disease advocates. According to the foundation, it is estimated 40,000 to 50,000 people are diagnosed annually with PF.

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