Sign up to receive more information about YUTREPIA

Sign Up

Understanding pulmonary hypertension associated with interstitial lung disease (PH-ILD)

 
  • Icon: Lung Illustration

    What is interstitial lung disease (ILD)?

    ILD is the name for a group of serious, progressive conditions, most of which cause scarring of lung tissue, making it harder to breathe and get enough oxygen into your bloodstream.

    Many different conditions are forms of ILD, including but not limited to:

    • Idiopathic pulmonary fibrosis (IPF) or pulmonary fibrosis (PF)
    • Combined pulmonary fibrosis and emphysema (CPFE)
    • Connective tissue disease (CTD)

    ILD is the name for a group of serious, progressive conditions, most of which cause scarring of lung tissue, making it harder to breathe and get enough oxygen into your bloodstream.

    Many different conditions are forms of ILD, including but not limited to:

    • Idiopathic pulmonary fibrosis (IPF) or pulmonary fibrosis (PF)
    • Combined pulmonary fibrosis and emphysema (CPFE)
    • Connective tissue disease (CTD)
  • Icon: PH in Lung Illustration

    What is pulmonary hypertension (PH)?

    PH is a serious and progressive condition in which there is high blood pressure in the arteries of the lungs and the right side of the heart. People with ILD are often at high risk of developing PH, known as pulmonary hypertension associated with interstitial lung disease (PH-ILD).

    PH is a serious and progressive condition in which there is high blood pressure in the arteries of the lungs and the right side of the heart. People with ILD are often at high risk of developing PH, known as pulmonary hypertension associated with interstitial lung disease (PH-ILD).

Illustration explaining how PHILD is a disease where there is high blood pressure in the arteries of your lungs, which causes the heart to work harder, becoming enlarged and weakened
 

What are the signs and symptoms of PH-ILD?

Many symptoms of PH are very similar to that of ILD, including shortness of breath, dizziness, fatigue, and chest discomfort.

  • Symptoms of PH may develop slowly over time and can add to the overall burden of the symptoms of ILD
  • After you have been diagnosed with ILD, it is important to be screened regularly for PH so you can receive appropriate treatment

Symptoms of PH-ILD include:

Illustration of Shortness of Breath

Shortness of Breath

Illustration of Tiredness

Tiredness

Illustration of Chest Pain or Pressure

Chest Pain or Pressure

Illustration of Fainting

Fainting

Illustration of Dizziness

Dizziness

Illustration of Rapid Heartbeat

Rapid Heartbeat

Illustration of Swollen Abdomen

Swollen Abdomen

Illustration of Swollen Legs and Ankles

Swollen Legs and Ankles

 

How is PH-ILD diagnosed?

Your doctor may perform several tests to determine if you have developed PH. Some of these tests may already be done regularly as a part of the routine monitoring of your ILD.

  • Electrocardiogram (EKG or ECG) icon

    Electrocardiogram (EKG or ECG) to measure the electrical activity of your heart

  • Pulmonary function test (PFT) icon

    Pulmonary function test (PFT) to see how well your lungs are working

  • 6-minute walk test (6MWT) icon

    6-minute walk test (6MWT) to see how far you can walk in 6 minutes

  • Echocardiogram (echo) icon

    Echocardiogram (echo) to check the size and function of your heart

  • Chest x-ray and ventilation perfusion scan (VQ scan) icon

    Chest x-ray and ventilation perfusion scan (VQ scan) to get images of your heart, lungs, and blood vessels

  • Blood tests icon

    Blood tests to check your oxygen levels and signs of liver, kidney, or heart problems

  • Right heart catheterization (RHC) icon

    Right heart catheterization (RHC) to measure the pressure inside your heart and the blood vessels of your lungs

RHC IS THE MOST CONCLUSIVE WAY TO DIAGNOSE PH.
 

How are the effects of PH-ILD on everyday activities measured?

A functional class assessment can help your doctor understand how much PH-ILD affects your everyday activities.

The functional classes are defined as follows:

NYHA Functional Class I No limitations on physical activity. Ordinary physical activity doesn't cause shortness of breath, fatigue, chest pain, or near fainting.
NYHA Functional Class II Some limitations on physical activity. Although comfortable at rest, ordinary activity causes shortness of breath, fatigue, chest pain, or near fainting.
NYHA Functional Class III Clear limitations on physical activity. Although comfortable at rest, even less than ordinary activity causes shortness of breath, fatigue, chest pain, or near fainting.
NYHA Functional Class IV Any level of physical activity is uncomfortable. There may be signs of heart failure, and shortness of breath and/or fatigue may be present even when resting.

NYHA = New York Heart Association

 

What treatment is available for PH-ILD and how does it work?

Inhaled prostacyclin therapy is the only FDA-approved type of treatment for PH-ILD

  • Prostacyclin is a natural substance that helps keep your pulmonary arteries open
  • Currently, the only approved prostacyclin therapy is called treprostinil, which works by acting like the prostacyclin produced in your body
Pulmonary Lung Illustration demonstrating Prostacyclin therapy

With inhaled treprostinil solution, most patients with PAH* or PH-ILD were able to walk further in a 6-minute walk test.

*In the clinical study for PAH, patients were also receiving up to 2 non-prostacylcin oral therapies as part of their overall treatment.