November is Pulmonary Hypertension (PH) Awareness Month, which is an opportunity to shine a light on this severe condition and share critical information on how it affects people living with scleroderma.
PH is a general term for high blood pressure in the arteries of the lungs. It can have several causes, and doctors group it into types based on what’s causing it. PAH is one type of PH, and it is the type most closely linked to scleroderma because it’s the only one caused directly by the disease itself. PAH affects 8% to 12% of people living with scleroderma.
Everyone diagnosed with scleroderma should be screened for PAH. This screening includes an echocardiogram (heart ultrasound), pulmonary function tests, and questions about shortness of breath. This screening should happen when a patient is first diagnosed and then every year after.
Pulmonary function test results can help doctors decide who needs more testing. If the ratio between two lung function measurements, forced vital capacity (FVC, how much air you can forcefully exhale) and diffusing capacity of the lungs for carbon monoxide (DLCO, how well oxygen passes from your lungs into your blood), is too high, doctors recommend a referral for right heart catheterization. This is a procedure where a thin tube is guided into the heart to measure blood pressure directly. It is the only test that can definitively confirm a PAH diagnosis.
A blood marker called NT-proBNP has also been linked to the development of PAH in scleroderma patients, and doctors now use it as part of screening tools to help identify who may need further testing.
Early detection means earlier treatment. This PH Awareness Month, ask your care team whether your annual PAH screening is up to date, and share this post to help spread the word.