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Why Is Learning About PH-ILD Important?

What Is PH-ILD?
Impact of PH-ILD
Detecting PH-ILD
Importance of a PH Specialist

Does your lung disease mean you are at risk for another condition?

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.

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Types of ILDs

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

  • Idiopathic nonspecific
  • Respiratory bronchiolitis-ILD
  • Desquamative
  • Cryptogenic organizing
  • Acute

Autoimmune diseases

  • Sarcoidosis
  • Rheumatoid arthritis
  • Sjogren’s syndrome
  • Lupus

What is PH-ILD?

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.

How do PH and ILD affect the lungs?

Learn how both PH and ILD affect the lungs, but in different ways, and how PH weakens the heart.

lung showing healthy air sacs versus alveoli damaged by interstitial lung disease

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

ILD

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 lung tissue in the spaces between air sacs is damaged, causing scarring and inflammation
  • The walls of the air sacs become inflamed, making it harder for the air sacs to fill with air and exchange oxygen with nearby blood vessels
  • Too little oxygen and excess scarring can lead to damaged or narrowed blood vessels
  • Breathing becomes more difficult, and other symptoms may develop

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.

PH

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:

  • Blood vessels in the lungs thicken and the passages where blood flows become narrower
  • Blood pressure within the vessels increases as less blood is able to flow through, and the heart and vessels become strained
  • The high blood pressure in the lungs can weaken the heart, causing difficulty breathing and other symptoms. This can cause heart failure over time
Thickened blood vessels in a lung affected by pulmonary hypertension

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.

Detecting PH early can be crucial to care management

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.

Watch for worsening ILD symptoms that may be a sign that you have developed PH

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

  • Fatigue
  • Shortness of breath
  • Reduced exercise ability

ILD symptoms

  • Cough
  • Weakness
  • More frequent colds and lung infections
  • Poor appetite/Weight loss
  • Reflux disease
  • Joint pain/stiffness
  • Rashes
  • Changes in fingernail shape

PH symptoms

  • Worsening symptoms over a short period
  • Increased need for supplemental oxygen
  • Palpitations
  • Chest pressure and/or pain
  • Dizziness or fainting
  • Swelling in legs or ankles
  • Inability to “recover” after exercise

PH screening is essential to disease monitoring

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.

See your healthcare team regularly

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.

Tests to assess for PH

Your healthcare team may use several tests to monitor for PH, including:

  • Preliminary tests that help evaluate the lungs—like exercise tests and other lung function tests—and special blood tests that evaluate your heart to see if it is strained
  • Echocardiogram (Echo) helps establish the need for further assessment
  • Right heart catheterization, which holds the key to diagnosing PH

Doctor Discussion Guide

Doctor Discussion Guide thumbnail

Keep asking questions and learning. This guide will help you partner with your healthcare team for better dialogue and care.

Download PDF

Symptom & Visit Tracker

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Use this tracker during and between visits to record vital information. Be sure to share it with your healthcare team at appointments.

Download PDF

Right Heart Catheterization FAQs

Right Heart Catheterization thumbnail

Learn more about the procedure used to diagnose PH

Download pdf

Importance of a PH Specialist

Recognizing 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 Specialist

You play an important role in your care management plan

PH 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:

  • Diet adjustments
  • Exercises or cardiopulmonary therapy
  • Use of supplemental oxygen

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 Group

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