Call the OPEN DOORS® Patient Support Program Care Team 24/7 1‑866‑673‑6366
Understanding your
Condition
If you or someone you care for has been recently diagnosed with interstitial lung disease (ILD), you may be wondering, “What happens now?” We are here to help. Learn more about this diagnosis, how ILD may affect the lungs, and why treating it may be an important step.
The more you know, the more empowered you and your loved ones will be to make important decisions about your care. Scroll down to learn more.
On This Page
Quick Fact: More than 200 types of ILD have been identified.
WHAT
IS ILD?
Interstitial lung disease, also known as ILD, refers to a large group of more than 200 disorders. It describes the development of inflammation and/or scarring around the air sacs of the lungs called the alveoli. These lung disorders may produce different levels of scarring, also called lung fibrosis.
ILD may be caused by a variety of potential factors, including autoimmune diseases and/or exposure to harmful substances like asbestos or silica dust, and medications. It can also be caused by many unknown factors.
Quick Fact: Lung fibrosis is also referred to as pulmonary fibrosis.
ABOUT YOUR
DIAGNOSIS
For some, the lung fibrosis caused by ILD may get worse over time and is believed to be irreversible. This worsening or “progressive” scarring can make it harder for the lungs to work properly, resulting in lung function decline. As every diagnosis is different, partnering with your doctor to determine how your body may be affected by lung scarring is a key step in your treatment plan.
Keep in mind, not all types of ILD with fibrosis progress—and some forms are more likely to produce this type of scarring than others. Let’s take a closer look at what you may be up against.
IPF is one of the most common forms of ILD with fibrosis. It is a serious and progressive disease, meaning it gets worse over time. It causes permanent scarring of the lungs, which results in impaired lung function. IPF is also unpredictable—no one knows who will get it or how quickly it will progress.
Idiopathic means the cause of the disease is unknown
Pulmonary means it impacts the lungs
Fibrosis means scarring
Quick Fact: For people with IPF, it is
important to remember that lung fibrosis is always progressive.
Learn
more about progression.
A chronic ILD with worsening fibrosis is a type of ILD in which lung fibrosis progresses, or continues to get worse over time. Although more than 200 different types of ILD have been identified, not all types are progressive.
Some examples of ILDs that are more likely to produce progressive scarring include:
- Hypersensitivity pneumonitis
- Unclassifiable ILDs
- Autoimmune ILDs, such as rheumatoid arthritis-associated ILD (RA-ILD), scleroderma-associated ILD, or myositis-associated ILD
- Occupational exposure-related ILDs
- Idiopathic pulmonary fibrosis (IPF)
- Idiopathic nonspecific interstitial pneumonia (iNSIP)
- Sarcoidosis-ILD
Quick Fact: It’s important to work with your doctor to determine how your lungs may be affected by ILD and if treatment might be right for you. Learn more about progression.
Scleroderma, also called systemic sclerosis or SSc, is a chronic condition that is caused when the immune system attacks the body’s own connective tissue. Scarring, also called fibrosis, is a key characteristic of the disease.
Scleroderma may cause extreme amounts of scarring, resulting in hard, thickened skin on the hands and other areas of the body. This scarring may affect multiple organ systems, including the lungs.
ILD is a common complication of the disease. When scleroderma affects the lungs, it is known as systemic sclerosis ILD (SSc-ILD) or scleroderma-associated ILD. The effects of scleroderma-associated ILD can vary from person to person. Its overall impact may range from mild to very serious.
Quick Fact: It is important to see your doctor regularly to test your lung function since lung fibrosis can develop rapidly and may continue to spread in your lungs. Learn more about monitoring your condition.
Idiopathic Pulmonary Fibrosis (IPF)
IPF is one of the most common forms of ILD with fibrosis. It
is a serious and progressive disease, meaning it gets worse over time. It
causes permanent scarring of the lungs, which results in impaired lung function. IPF
is also unpredicatable—no one knows who will get it or how quickly it will progress.
Idiopathic means the cause of the disease is unknown
Pulmonary means it impacts the lungs
Fibrosis means scarring
Quick Fact: For people with IPF, it is important to remember that lung fibrosis is always progressive. Learn more about progression.
Chronic ILDs with Worsening Fibrosis
A chronic ILD with worsening fibrosis is a type of ILD in which lung fibrosis progresses, or continues to get worse over time. Although more than 200 different types of ILD have been identified, not all types are progressive.
Some examples of ILDs that are more likely to produce progressive scarring include:
- Hypersensitivity pneumonitis
- Unclassifiable ILDs
- Autoimmune ILDs, such as rheumatoid arthritis-associated ILD (RA-ILD), scleroderma-associated ILD, or myositis-associated ILD
- Occupational exposure-related ILDs
- Idiopathic pulmonary fibrosis (IPF)
- Idiopathic nonspecific interstitial pneumonia (iNSIP)
- Sarcoidosis-ILD
Quick Fact: It’s important to work with your doctor to determine how your lungs may be affected by ILD and if treatment might be right for you. Learn more about progression.
Scleroderma-associated ILD
Scleroderma, also called systemic sclerosis or SSc, is a chronic condition that is caused when the immune system attacks the body’s own connective tissue. Scarring, also called fibrosis, is a key characteristic of the disease.
Scleroderma may cause extreme amounts of scarring, resulting in hard, thickened skin on the hands and other areas of the body. This scarring may affect multiple organ systems, including the lungs.
ILD is a common complication of the disease. When scleroderma affects the lungs, it is known as systemic sclerosis ILD (SSc-ILD) or scleroderma-associated ILD. The effects of scleroderma-associated ILD can vary from person to person. Its overall impact may range from mild to very serious.
Quick Fact: It is important to see your doctor regularly to test your lung function since lung fibrosis can develop rapidly and may continue to spread in your lungs. Learn more about monitoring your condition.
HOW ILD
MAY AFFECT THE LUNGS
While the causes of different types of ILD with fibrosis may vary widely, how they affect the lungs is very similar.
Scar tissue forms in the walls of the alveoli in the lungs. The stiff scar tissue makes it harder for oxygen to pass through the alveoli walls and into the bloodstream and for carbon dioxide to be removed. Over time, this scarring may get worse, affecting lung function and making it harder to breathe.
Quick Fact: The damage progressive lung fibrosis causes is believed to be irreversible. Starting treatment is important to help slow lung function decline.
Learn more about how ILD with fibrosis may affect your lungs.