Interstitial lung diseases are characterized by changes in the air sacs, such as scarring, inflammation or both. Interstitial lung diseases can be caused by autoimmune conditions or exposure to certain medicines, dust, chemicals, mold, smoke or other inhaled substances. These lung diseases can lead to Group 3 pulmonary hypertension for a variety of reasons, including a stiff left side of the heart or a lack of oxygen.
Idiopathic pulmonary fibrosis is the most common type of interstitial lung disease. Idiopathic means without a known cause and fibrosis means scarring. Pulmonary fibrosis is characterized by scarring in the lungs that continues to get worse.
Patients often complain of breathlessness (especially with activity), dry cough and fatigue. Pulmonary fibrosis is characterized by scarring in the lungs that continues to get worse.
Diagnosis and treatment usually take place at a center specializing in interstitial lung disease. The results of a high resolution CAT scan, along with blood work findings, determine whether a lung biopsy is necessary.
Some patients with pulmonary fibrosis develop PH, which can worsen shortness of breath and cause dizziness.
Diagnosing PH
About one in 20 patients or 5% of patients who are seeing a doctor for the first time for pulmonary fibrosis may also have pulmonary hypertension. Many more may develop PH, especially if they have also have emphysema. Patients with interstitial lung disease can develop PH for many reasons. It’s important to determine the root cause of PH for each patient.
Diagnostic tests
Tests to determine PH may include:
- Echocardiogram
- Pulmonary function tests
- Ventilation-perfusion (V/Q) scan
- Natriuretic peptide tests
- Right heart catheterization
Diagnostic tests will measure pressures in the heart and lungs, and a right heart catheterization can also determine whether patients have problems with a stiff left side of the heart. Left heart disease also can cause PH. In those cases, doctors refer patients to a cardiologist to treat the heart disease.
For people with interstitial lung disease, it’s important to rule out obstructive sleep apnea through a sleep study, as sleep apnea can cause PH.
If doctors can’t identify other causes of PH, they can diagnose PH due to the patient’s interstitial lung disease.
FDA-approved treatment
Inhaled treprostinil is an FDA-approved drug to improve exercise capacity and treat ILD-associated PH. It is available in three formulas: Tyvaso, Tyvaso DPI and Yutrepia.
All PH treatments
People with PH-related to idiopathic pulmonary fibrosis often have low oxygen levels with exercise and at night, so those people need to use oxygen during sleep and with activity.
Supplemental OxygenIt is important to get treatment for interstitial lung disease. The Food and Drug Administration has approved several medications to treat pulmonary fibrosis and other fibrotic interstitial lung diseases. PHA and the Pulmonary Fibrosis Foundation clinicians collaborated on guidance to treat PH related to interstitial lung disease.
Pulmonary Hypertension related to Interstitial Lung DiseaseTransplantation is an option for some people with advanced interstitial lung disease. Patients need to be evaluated at a transplant center to determine whether they are candidates for lung transplant. For people deemed appropriate for lung transplant, PH can trigger placement on the transplant waiting list.
TransplantationIt is important to stay as active as possible. A monitored exercise program called pulmonary rehabilitation can decrease symptoms and improve endurance for people with PH and interstitial lung diseases. Pulmonary rehabilitation takes place on an outpatient basis at a hospital and requires referral by a treating pulmonologist. A monitored exercise program called pulmonary rehabilitation can decrease symptoms and improve endurance for people with PH and interstitial lung diseases.
ExercisePeople with PH-related to idiopathic pulmonary fibrosis often have low oxygen levels with exercise and at night, so those people need to use oxygen during sleep and with activity.
It is important to get treatment for interstitial lung disease. The Food and Drug Administration has approved several medications to treat pulmonary fibrosis and other fibrotic interstitial lung diseases. PHA and the Pulmonary Fibrosis Foundation clinicians collaborated on guidance to treat PH related to interstitial lung disease.
Transplantation is an option for some people with advanced interstitial lung disease. Patients need to be evaluated at a transplant center to determine whether they are candidates for lung transplant. For people deemed appropriate for lung transplant, PH can trigger placement on the transplant waiting list.
It is important to stay as active as possible. A monitored exercise program called pulmonary rehabilitation can decrease symptoms and improve endurance for people with PH and interstitial lung diseases. Pulmonary rehabilitation takes place on an outpatient basis at a hospital and requires referral by a treating pulmonologist. A monitored exercise program called pulmonary rehabilitation can decrease symptoms and improve endurance for people with PH and interstitial lung diseases.
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Information on PH and interstitial lung disease is one of a series of PH& brochures available to download as a PDF or to order as a printed brochure.
PH & ILD
