If you have been diagnosed with an interstitial lung disease (ILD), you may also be at risk of developing another condition called pulmonary hypertension (PH)—which is why asking your healthcare provider about testing for PH is so important. Keep reading to see some of the lung diseases that are classified as ILDs and learn about their symptoms, including those that overlap with PH symptoms. You’ll also discover why PH screenings are important for people who have an ILD.
Hundreds of lung conditions are classified as ILDs. Some of the more common ILDs are shown below.
Idiopathic pulmonary fibrosis
A type of interstitial pneumonia and the most common ILD
Other interstitial pneumonias
Autoimmune diseases
When a person with an ILD develops PH, the condition is known as PH-ILD.
Both PH and ILD damage the lungs, and PH also affects the heart. PH can appear at any stage of ILD and can impact life expectancy. That’s why early detection of PH is so important.
Learn how both PH and ILD affect the lungs, but in different ways, and how PH weakens the heart.
Healthy (left) vs inflamed/scarred (right) air sacs
Cross sections show how the shape of air sacs, and their ability to expand, can be restricted by inflammation and/or scarring
affects air sacs in the lungs
ILDs damage tissue and air sacs (alveoli) inside your lungs. As time passes, the damage worsens and can cause harmful scarring. This damage can limit how much oxygen will get into your bloodstream and reach the rest of your body.
In people who don’t have an ILD, air sacs (alveoli) expand to fill with air, which promotes healthy breathing.
In people who have an ILD:
The changes in your lungs that are caused by ILD may eventually damage or narrow your blood vessels, which causes additional shortness of breath and strain on your heart.
affects blood vessels in the lungs
PH is high blood pressure that affects the blood vessels in your lungs. If you have PH, your heart has to work harder to pump blood through the vessels, and it grows weak. This results in less blood flow and less oxygen available to reach the rest of the body.
In people who don’t have PH, blood vessels in the lungs are open and wide, allowing blood to pass through easily.
In people who have PH:
Healthy (left) vs thickened (right) blood vessels
Cross sections of blood vessels show how the thickening of the vessel wall narrows the inside of the vessel, allowing less blood to flow through
Developing PH may be a sign that an ILD will progress more quickly.
PH and ILD affect the lungs in different ways, but their symptoms can be similar. If you have an ILD, paying close attention to symptom worsening or an increased use of oxygen is essential because it can provide important clues that you might have developed PH. If you notice a change in symptoms or your ability to do exercises, tell your healthcare team right away and ask about testing for PH. Now more than ever, we are learning more about PH-ILD care, so keeping your healthcare team updated about any changes in your symptoms is key.
PH can be a serious condition by itself—and when people who already have an ILD develop PH, it can make the ILD worsen more quickly. Having PH in addition to an ILD usually makes breathing more difficult and often requires an increase in supplemental oxygen.
PH-ILD overlapping symptoms
ILD symptoms
PH symptoms
Every visit with your healthcare team is an opportunity to ask if changes in your symptoms or test results could indicate PH, so you can get the answers and follow-up care you need.
Managing your symptoms is important to feel the best you can. Discussing your symptoms with your doctor makes a difference and can ultimately lead to earlier detection of PH-ILD.
Your healthcare team may use several tests to monitor for PH, including:
Doctor Discussion Guide
Keep asking questions and learning. This guide will help you partner with your healthcare team for better dialogue and care.
Download PDFSymptom & Visit Tracker
Use this tracker during and between visits to record vital information. Be sure to share it with your healthcare team at appointments.
Download PDFRecognizing PH-ILD early may change how your care is managed. Pulmonologists who specialize in both PH and ILD are rare. To receive the best care, you may need to see more than one specialist—one for PH and another for ILD.
In addition to asking your healthcare team if an assessment for PH may be appropriate, consider inquiring about what specialist(s) might join the team if you have PH-ILD.
Find a PH SpecialistPH can develop at any time during the course of an ILD. If you develop PH-ILD, it’s up to you to stay on top of any changes to your care plan that your healthcare team may suggest, which could include:
Address all your questions and concerns during visits with your healthcare team–you might ask about available clinical trials.
Seek support in your everyday life, too, from loved ones and others who are dealing with similar conditions.
Find a Support GroupContinue monitoring for PH-ILD signs and symptoms and learning all you can about your diagnosis and condition.
Get PH-ILD updates
