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Idiopathic Pulmonary Fibrosis, a Diagnosis with Worse Outcome Than Some Cancers

By Johanna Elizabeth Ku posted 3 hours ago

  
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In our modern lives, we are running from one thing to another. To calm down, we tend to start breathing calmly and in a controlled way. Our lungs fill with air, and in our alveoli, oxygen is exchanged for carbon dioxide. Our heartbeats slow, and our blood pressure decreases. After a couple of minutes, we move on to the next appointment.

We often do not think about the important role our lungs play in our daily lives until we get sick and we can´t breathe normally. For most of us, we will be able to recover… but some will start experiencing a cough, dyspnea (shortness of breath and fatigue) that will turn their lives around due to an unexpected diagnosis.

During the Workshop Session “Environmental Exposures and Pulmonary Fibrosis: What Do We Know and Where Do We Go?”, the presenters explained that interstitial lung disease (ILD) is an “umbrella term” for more than 200 diseases characterized by inflammation and/or scarring of the lung parenchyma, the lung structures that primarily support gas exchange. Certain IDL subtypes are exposure-related, meaning the cause can be traced to viral infection, smoking, air pollution, or occupational exposure, among other factors. If exposure is causing inflammation, treatment can improve it. But if the cause can´t be determined and the lung is suffering irreversible scarring in the lung parenchyma, the person is diagnosed with idiopathic pulmonary fibrosis (IPF). IPF is the most common type of ILD where the therapeutics are to slow down the progression of scarring. Unfortunately, there is no cure, and patients have a worse prognosis and survival rate than many common cancers: the patient's prognosis is three to five years post-diagnosis, where only 20% of patients will survive beyond five years after diagnosis.

It is estimated that, worldwide, 6six million patients are diagnosed each year, and 200,000 cases are diagnosed in the United States. Unfortunately, air pollution is a product of our industrial lifestyle and will worsen as the climate warms, leading to more cases. It is hard to define a “sale level or air pollution” due to many personal variations (like genetic predispositions). During the panel discussion, someone asked how we can all work together to reach a consensus on answers to some research questions. The following ideas were shared:

  • It is necessary to define which are the key central pathways for ILDs and IPF.

  • It is necessary to recognize the difference between exacerbation of pulmonary fibrosis (AE-PF) and incidence of IPF. It is possible that some processes might coincide, but they should be seen as two separate processes.

  • It is necessary to understand the coordinated response that exists when the body recognizes that the lung has been injured or irritated. How does that immune response act in a coordinated fashion to return to a homeostatic state?

  • We need to understand which are the pathways that can be used to develop new drugs or interventions?

Finally, if you are interested in reading more about idiopathic pulmonary fibrosis (IPF), these were three review articles cited by the presenters:

  • Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020 Sep 3;383(10):958-968. doi: 10.1056/NEJMra2005230. PMID: 32877584.

  • Pardo A, Selman M. The Interplay of the Genetic Architecture, Aging, and Environmental Factors in the Pathogenesis of Idiopathic Pulmonary Fibrosis. Am J Respir Cell Mol Biol. 2021 Feb;64(2):163-172. doi: 10.1165/rcmb.2020-0373PS. PMID: 32946290.

  • Michalski JE, Schwartz DA. Genetic Risk Factors for Idiopathic Pulmonary Fibrosis: Insights into Immunopathogenesis. J Inflamm Res. 2021 Jan 5;13:1305-1318. doi: 10.2147/JIR.S280958. PMID: 33447070; PMCID: PMC7801923.

This blog reports on the Workshop Session titled “Environmental Exposures and Pulmonary Fibrosis: What Do We Know and Where Do We Go?” that was held during the 2026 SOT Annual Meeting and ToxExpo. An on-demand recording of this session is available for meeting registrants on the SOT Online Planner and SOT Event App.

This blog was prepared by an SOT Reporter and represents the views of the author. SOT Reporters are SOT members who volunteer to write about sessions and events in which they participate during the SOT Annual Meeting and ToxExpo. SOT does not propose or endorse any position by posting this article. If you are interested in participating in the SOT Reporter program in the future, please email SOT Headquarters.


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