Interstitial Lung Disease

Interstitial lung disease (ILD) is a group of conditions involving scarring of the tissue around the air sacs in the lungs. Only a lung transplant can restore function, but these are rare. Treatments focus on slowing the progression of the disease and relieving symptoms.

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What Is Interstitial Lung Disease?

Interstitial lung disease is not one specific illness or condition; instead, it is a category of more than 100 lung diseases with various possible causes, though sometimes the cause is never determined.

Because there are so many ILDs, they are generally categorized by association with a certain disease or environmental contaminant.[1] For example, ILD caused by asbestos exposure is called asbestosis. Asbestosis belongs to the exposure category of ILDs.[2]

All ILDs cause lung tissue scarring that worsens over time. ILDs are specifically characterized by scarring around the alveoli or air sacs of the lungs, also known as the interstitium, or interstitial space.

The interstitium is important for the exchange of oxygen and other gases between the lungs and capillaries. It becomes progressively more difficult for this exchange to occur as scarring worsens.

Scarring Caused by Asbestos

Interstitial lung disease caused by exposure may occur when someone inhales airborne contaminants. Possible contaminants include silica, coal dust, farm dust, fumes from welding, and much more. For someone who has worked around asbestos, inhalation of the fibers may lead to lung scarring that causes asbestosis.[1]

When asbestos fibers enter the lungs, they can become embedded in tissue. Sometimes this occurs in the pleura and may lead to mesothelioma. These tiny fibers can also travel further into the lungs, causing damage to tissue in the interstitium.

In response to this damage, the body attempts to make repairs, thickening tissue and causing scarring. The scarring is more extensive with greater exposure to asbestos. Unfortunately, even when the contaminant is removed, scarring continues to worsen.

Symptoms of ILD

While various contaminants may cause an ILD through exposure, they all cause similar symptoms. These symptoms are also similar to other lung conditions, like chronic obstructive pulmonary disease (COPD) and asthma.

Symptoms of ILD include:[3]

• Shortness of breath
• Chest pain
• Coughing
• Fatigue that worsens with exercise or exertion
• Unexplained weight loss

Once symptoms are obvious, the disease has likely progressed so far that it is irreversible.[2]

Diagnosis

Diagnosing an ILD can be tricky because symptoms are similar to other more common conditions, like COPD. If you have been exposed to asbestos, dust, or environmental contaminants, it is important to inform your doctor to receive the most complete diagnosis. These are the typical steps in a diagnosis:[4]

• Diagnosis generally begins with chest X-rays and lung function tests.
• Lung function tests determine if your lung capacity is diminished.
• Other imaging tests, like CT scans or MRIs, may also be used to provide a more detailed picture of tissues in your chest cavity and lungs.
• In some cases, a biopsy will be performed. For a biopsy, a small sample of tissue is removed and examined by a pathologist.
• Pathologists use the tissue sample to determine if the tissue is scarred by ILD.

Treatment for ILDs

It is important to find the underlying cause of an ILD to determine the best course of treatment. If your ILD is caused by asbestos, you will need to avoid any further exposure.

Treatment for ILDs also includes procedures to treat scarring, limit further damage, and help your lungs get more oxygen.[4]

Medications may help slow the formation of scar tissue; however, they are not always effective depending on the underlying cause of the ILD.

Oxygen therapy will not slow damage, but it can help you breathe more easily, increase your oxygen intake, and help you sleep better. Oxygen therapy involves supplemental oxygen typically administered through an oxygen tank.[4]

In rare cases, a lung transplant may be an option, when damage to lung tissue is severe and the patient is otherwise healthy.

Lung transplants are not common for treating asbestosis; however, if a lung is available and a patient is a good candidate, doctors may consider this difficult procedure. For some patients, a transplant is the best option though it is risky and may result in organ rejection.[4]

Complications of Asbestosis and other ILDs

In addition to difficulty breathing and inadequate oxygen, ILDs can cause other serious complications. High blood pressure in the lungs, or pulmonary hypertension, can be triggered by an ILD.

These conditions can be life-threatening. If you have asbestosis and you smoke, you may also be at risk for lung cancer. ILDs may also cause pleural plaques and pleural effusions, the thickening of lung tissue, and fluid buildup around the lungs.

Prognosis

If you are diagnosed with asbestosis or another ILD, your prognosis will depend on the extent of your disease. If caught early, asbestosis may not cause serious symptoms or complications.

Early treatment can mitigate symptoms and slow progression. When not diagnosed until extensive damage has been done, or if you also smoke, the prognosis will likely be worse.

ILDs caused by exposure are preventable. If you work around harmful chemicals, minerals, or dust, be sure to use proper protective equipment. This is an important step in preventing damage to your lung tissue. Living with ILD is uncomfortable, limiting, and may ultimately cost you your life.

Page Medically Reviewed and Edited by Anne Courtney, AOCNP, DNP

Anne Courtney has a Doctor of Nursing Practice degree and is an Advanced Oncology Certified Nurse Practitioner. She has years of oncology experience working with patients with malignant mesothelioma, as well as other types of cancer. Dr. Courtney currently works at University of Texas LIVESTRONG Cancer Institutes.

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