Pulmonary Fibrosis Foundation and Pulmonary Hypertension Association Provide Guidance on Pulmonary Hypertension Related to Interstitial Lung Disease

Pulmonary Hypertension Interstitial Lung Disease

The Pulmonary Fibrosis Foundation (PFF) and Pulmonary Hypertension Association have developed a position statement that addresses gaps in the screening, diagnosis and treatment of patients living with pulmonary hypertension (PH) related to interstitial lung disease (PH-ILD). The PH-ILD position statement, featuring a version for patients and a version for healthcare providers, reflects input from experts in interstitial lung disease (ILD), PH, PH-ILD and patient representatives.

More than 250,000 Americans are living with pulmonary fibrosis (PF) and ILD. PF, a form of ILD, can have many causes and they can all be associated with PH. PF causes chronic damage in the tissues of the lungs. When PF is more severe, there is a greater chance of developing PH, which is defined as high blood pressure in the lungs and can lead to right heart failure.

People living with PH-ILD have a greater symptom burden and worse outcomes compared to those with interstitial lung disease alone, and until recently, there have been no effective or approved treatments for these patients,” said Dr. Franck Rahaghi, President, CEO and CMO for the PFF. “The goal of our position statement is to raise awareness of PH-ILD and to share the latest knowledge on the diagnosis and treatment of this complex condition.”

“Pulmonary fibrosis and ILD are two of many conditions and diseases associated with a higher risk of PH, which can severely affect a person’s quality of life,” said Matt Granato, President and CEO of PHA. “These statements should help facilitate early diagnosis and treatment, which is key to improving outcomes for PH-ILD patients.”

The statement advises that it is important to screen ILD patients for PH early after ILD diagnosis. Worsening breathing symptoms, physical exam findings, oxygen levels, or changes in breathing tests and/or walking tests should prompt consideration of and evaluation for PH-ILD. While non-invasive tests can help to screen for the condition, right heart catheterization is necessary for making the diagnosis of PH-ILD.

The statement also provides information on therapies for PH-ILD, including pulmonary vasodilator medication, treatment of the underlying disease, and supportive interventions, such as supplemental oxygen and lung transplantation.

For patients seeking more information on PH-ILD, the position statement is available here. Healthcare providers can find more comprehensive information here.


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