Rheumatologist Calls for More Screening of Scleroderma Patients for Pulmonary Hypertension

Many systemic sclerosis patients develop pulmonary arterial hypertension, but not all who are suspected of having PAH are referred for testing, according to rheumatologist Janet Pope.

She called for more commitment to screening in an editorial in Arthritis Research & Therapy. The piece was titled “Pulmonary arterial hypertension in scleroderma: care gaps in screening.”

PAH is a lung disease marked by high blood pressure in pulmonary arteries. It can lead to heart failure and death.

Fifteen percent of patients with systemic sclerosis, also known as SSc or scleroderma, develop pulmonary hypertension, Pope said.

“Screening is important because there are no reliable risk factors” for scleroderma patients developing PAH, Pope wrote.

“Age is a likely risk,” she said. The older people are when they develop scleroderma, the more likely they will develop PAH, she said. Another likely risk factor is duration of scleroderma, with those having it longer more likely to develop PAH, she said.

Low lung diffusion capacity, or inability to process oxygen properly, “is associated with current and/or future PAH,” Pope said. “However, this is likely not a risk factor” for the disease. Instead, it’s a sign that PAH may be developing.

There are a number of preliminary PAH screening tests, including some that provide information on pulmonary function. Results of these tests give doctors an idea of which patients should have an echocardiogram, a more definitive test that measures pulmonary artery pressure.

Patients with no PAH symptoms, such as shortness of breath, may not benefit from screening. But some scleroderma patients develop PAH without experiencing this problem, known as dyspnea.

“Even if someone is very active and has no symptoms, results of an echocardiogram can help to predict future development of symptomatic PAH,” Pope wrote. “This needs to be balanced by the probability of false positive screening tests which may occur.” False positives are test readings that indicate a person has a disease when they do not.

Since scientists have yet to come up with a cure for PAH, regular screening and early diagnosis give patients the best chance to improve their symptoms and ultimately their survival rate.

“Screening for PAH in SSc allows for earlier detection and treatment that prolongs survival and improves symptoms,” Pope said. ” But it is important that clinicians [doctors] who follow SSc patients screen and act upon the results, such as referring suspected PAH for right-heart catheterization and treatment at an expert center.”