A primer on interstitial lung diseases for ILD Day
ILD Day aims to provide educational information and raise awareness
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Because idiopathic pulmonary fibrosis (IPF) is a diagnosis of exclusion, it’s easy to forget about all of the other related diseases that make up the interstitial lung disease (ILD) family, which includes more than 200 types. IPF, much like a family with a lot of cousins, is easier to understand when you know what those cousins contribute to your journey. What about the progression of another ILD, or what research into another ILD may contribute to a better understanding of IPF?
When I was diagnosed with IPF in January 2017, I had heard the term ILD during my journey. A patient with ILD can present with shortness of breath, a chronic cough, fatigue, and chest discomfort. Regardless of how the patient presents with ILD, it is an inflammation of the interstitium (the fluid-filled space between the cells and tissues in your body) and often results in scarring in the lungs.
An ILD, which is an obstructive disease, is not like chronic obstructive pulmonary disease or asthma, which are restrictive diseases.
Even as a diagnosis of exclusion, IPF is the most common ILD. Sometimes those diagnoses are wrong. When they are, it can be a relief, but the underlying condition may still exist.
When the cause of the inflammation and scarring is known, it may be due to exposure to asbestos, which results in a diagnosis of asbestosis. Exposure to asbestos fibers is not a thing of the past. A remediation program to remove asbestos from the Pentagon, one of the largest office buildings in the world, wasn’t completed until 2011, for example. The number of employees in the Pentagon alone creates a pool of potential patients exposed to asbestos.
Exposure to even small airborne particles can lead to a diagnosis of pulmonary fibrosis (PF). A 2016 report found that members of the dental profession in a specific dental center were being diagnosed with IPF. This is an example of workplace exposures that led to new respiratory protections.
I want to share three other ILDs: hypersensitivity pneumonitis, radiation pneumonitis, and nonspecific interstitial pneumonitis.
Chronic hypersensitivity pneumonitis results from continued or repeated exposures over time. The patient may be asked about exposure to water damage from a burst pipe or flood, or whether they have a hot tub at home. At some point, the patient may present with clubbed fingers, which can often lead to a diagnosis of PF.
Radiation pneumonitis typically occurs following radiation treatment for lung cancer. The incidence of radiation pneumonitis has decreased due to improved radiotherapy techniques for treating lung cancer, but it is not zero. Patients do not experience symptoms immediately. In some cases, it occurs months after radiotherapy.
Finally, there are two forms of nonspecific interstitial pneumonitis: cellular and fibrotic. The fibrotic form can lead to a diagnosis of PF, while the cellular form may be curable. Nonspecific interstitial pneumonitis is often associated with connective tissue diseases, such as scleroderma. When it affects the lungs, it can cause shortness of breath and lung scarring.
The first ILD Day in 2021 helped to raise awareness of these diseases. Today, ILD Day, observed on Sept. 16, is a collaboration of 13 organizations working together to provide educational information and raise awareness. The focus this year is precision medicine, highlighted by the question, “What if there was a treatment plan tailored to you?”
IPF is just one of many ILDs. Research on scarring in one ILD may apply to another. You can register for a free webinar on precision medicine and some of the factors considered when building a treatment plan. Knowledge is power, and I certainly want to know how I can make every breath count.
Note: Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Fibrosis News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to pulmonary fibrosis.
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