Interstitial Lung Disease (ILD)

The lungs are one of the most important organs in the body. When the lungs become heavily scarred due to exposure to toxins or because of congenital disorders, they lose the ability to function properly. Interstitial lung disease (ILD) refers to a wide range of conditions that cause scarring of the lungs, affecting a person’s ability to breathe and supply oxygen to their bloodstream.

The University of Kansas Health System offers comprehensive care for these rare diseases that can lead to pulmonary fibrosis. As a national center of excellence, we are home to one of the few programs of this type in the Midwest.

What is interstitial lung disease?

The interstitium is a part of the lungs that exists between the cell walls. It absorbs and processes air as you inhale and exhale. This part of the lungs serves as a scaffolding in which these cells sit and help to drain fluid that flows between them. When the interstitium becomes thickened due to scarring, inflammation or fluid buildup, the lungs are considered to be experiencing ILD.

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Types of interstitial lung disease

There are 2 broad types of ILD that a person may experience:

Acute ILD
Chronic ILD

Acute ILD refers to a condition in which damage to the interstitium is severe, but may last for only a short time.

Chronic ILD is a disease in which the condition of the interstitium gradually worsens and does not get better.

Both acute and chronic ILD can occur for a number of reasons, which are categorized based on the cause of the disease.

This is a rapid-onset and severe form of interstitial lung disease that typically requires immediate intervention through the use of a ventilator.
This form of interstitial lung disease is caused by breathing the building material asbestos; may present similarly to mesothelioma, a form of lung cancer caused by asbestos inhalation.
Similar to pneumonia, this form of ILD occurs without an infection and is often referred to as bronchiolitis obliterans with organizing pneumonia by pulmonary specialists.
This form of ILD most often occurs from smoking.
This form of ILD occurs from breathing irritants such as dust or mold over a long period of time.
Pulmonary fibrosis refers to a general scarring of the interstitium for reasons currently not known.
Microorganisms such as bacteria or fungi can infect the interstitium, leading to ILD.
Some people with autoimmune disorders such as rheumatoid arthritis or scleroderma are at risk for developing ILD.
This condition affects both the interstitium and the lymph nodes, and may include symptoms in the heart, nervous system, skin and eyes.

Interstitial lung disease symptoms and risks

Symptoms of ILD include difficulty breathing, shortness of breath and chronic cough. In more severe cases, the blood pressure in the lungs is heightened, which can lead to heart failure. Respiratory failure can also occur as a result of interstitial lung disease.

Because ILD impairs the body’s ability to transfer oxygen to the bloodstream, those with ILD may frequently feel very tired or fatigued, particularly after physical activity. Unexplained weight loss and pain in the chest are also symptoms of ILD.

Interstitial lung disease diagnosis and screening

A family history of ILD is a contributing factor to its formation, so the first step in diagnosis is an examination of your medical history, followed by a physical examination. Pulmonary function tests are commonly performed with the aid of specialized machines that measure the lungs' ability to properly function. These tests can include chest X-rays, CT scans and blood tests.

Spirometry is a common test that measures the lungs’ ability to function. This test uses a device called a spirometer, which can determine the lungs’ ability to take in, hold and expel air. Spirometry helps doctors determine the severity and type of problems experienced within the lungs, and is also often used throughout treatment to determine its effectiveness.

Leading research and clinical trials

As part of one of the nation's premier academic medical centers, our care providers are committed to research and scientific discovery through the University of Kansas Medical Center. We can often include our patients in potentially lifesaving clinical trials and treatment options not available anywhere else.

Our Research

Interstitial lung disease treatment

For people who smoke, the first stage of treatment is to quit smoking. Oxygen therapy and medication are also common treatments for ILD. Medications used to treat ILD include:

Antifibrotics to help prevent scarring
Corticosteroids to reduce inflammation
Cyclophosphamide to suppress the patient’s immune system

The most severe cases of interstitial lung disease may require a lung transplant.

Why choose us for interstitial lung disease care

The interstitial lung disease program at The University of Kansas Health System has been recognized as a center of excellence by the World Association for Sarcoidosis and Other Granulomatous Disorders, the Rare Lung Disease Consortium and the LAM Foundation. In addition, our IDL program has been included as part of the Pulmonary Fibrosis Foundation's Specialty Care Centers. This elite network of medical centers includes only those providers who:

Have expertise in treating adults with fibrotic lung diseases
Use a multidisciplinary approach to deliver comprehensive care
Engage in patient-oriented pulmonary fibrosis research
Work with the Pulmonary Fibrosis Foundation to provide educational materials and engage the local community through PFF programs

In 2014, our IDL program was among the first 20 of only 43 centers across the U.S. to earn this achievement.

We also offer dedicated ILD clinical trials, which gives patients the opportunity to benefit from advancements in medicine.

Your interstitial lung disease care team

Your interdisciplinary care team is comprised of pulmonologists with advanced training in interstitial lung disease, as well as dedicated respiratory therapists, thoracic radiologists, lung pathologists and rheumatologists. Together, these specialists offer unparalleled expertise in both diagnosis and treatment of IDL. They collaborate to ensure that you benefit from accurate diagnosis, comprehensive care, support services and the opportunity to participate in groundbreaking clinical trials.