Pulmonary hypertension (PH) is a rare condition that may arise as a complication of interstitial lung disease (ILD). Here are 5 things that might help if you have or think you might have PH related to ILD.

1: Pulmonary hypertension is the condition of having high blood pressure in the blood vessels that runs through the lungs. Blood is pumped from the right side of the heart through the lungs to pick up oxygen, then it goes back to the left side of the heart and is pumped out to the rest of the body to deliver that oxygen. Patients with pulmonary fibrosis (PF) or ILD can also develop PH.

2: There are 5 types of PH, and PH related to ILD falls into the third type or “group 3 PH”. This category of PH is related to structural problems with the lungs, including:

• PF and ILD or emphysema,
• and / or low oxygen levels.

The stiffness of the lungs and the low oxygen levels contribute to the increased blood pressure, which is normally low in the pulmonary blood vessels. The classification of a person’s PH is important to understand during the diagnostic process.

Bonus fact: Some patients are diagnosed with PH then found to have ILD as the cause during a thorough clinical evaluation, and other patients are diagnosed with ILD then develop PH later in the disease course.

3: Symptoms of PH related to ILD are often very similar to symptoms of ILD alone, including increasing shortness of breath, fatigue, and greater oxygen needs. Other symptoms that are usually not seen in ILD alone might be leg swelling or fluid retention, lightheadedness, or chest discomfort. It’s important to talk with your doctor about changes in symptoms or new symptoms that concern you.

4: Testing to look for PH related to ILD may include lab work and an echocardiogram, which is an ultrasound test that estimates heart function and pressures inside the heart. A right heart catheterization is required to confirm the diagnosis of PH related to ILD. This involves the placement of a catheter through a vein and into the right side of the heart to directly measure pressures and blood flow. It’s an outpatient procedure commonly done in cardiac catheterization labs, usually by a cardiologist but sometimes by a pulmonologist.

5: There are several medications that are FDA approved specifically for treatment of PH, but only one type of medication – inhaled treprostinil (brand names Tyvaso, Yutrepia) – is FDA approved for PH related to ILD. Treatment is intended to improve how a person feels and functions by lowering the blood pressure in the lung circulation. Other treatments include diuretic medications to help remove excess fluid from the body, pulmonary rehabilitation, supplemental oxygen, and lung transplantation.

Ready to learn more? Check out our position statement on Pulmonary Hypertension related to ILD, developed in collaboration with the Pulmonary Hypertension Association. Be sure to tune in to our July webinar to get more in depth.

About the Pulmonary Fibrosis Foundation
At the Pulmonary Fibrosis Foundation, we are dedicated to making a difference in the lives of those affected by pulmonary fibrosis (PF), a form of interstitial lung disease (ILD). Pulmonary fibrosis is a process that causes lung scarring, in which fibrotic tissue blocks the movement of oxygen from inside the tiny air sacs in the lungs into the bloodstream. Low oxygen levels, and the stiff scar tissue itself, can cause people with pulmonary fibrosis to feel short of breath, particularly when walking and exercising. Over 250,000 Americans are living with PF today. Approximately 50,000 new cases are diagnosed each year and as many as 40,000 Americans die from idiopathic pulmonary fibrosis (IPF) each year.
As the largest organization committed to raising awareness and providing support, our mission is to accelerate the development of new treatments and ultimately a cure for pulmonary fibrosis. Until this goal is achieved, the PFF is committed to advancing improved care of patients with PF and providing unequaled support and education resources for patients, caregivers, family members, and healthcare providers.