Patients with scleroderma should receive regular pulmonary arterial hypertension screening
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SAN DIEGO — Pulmonary arterial hypertension’s prevalence and rate of progression in patients with connective tissue diseases should make regular screening a priority, according to a speaker at the Congress of Clinical Rheumatology West.
“Screen your patients. Screen all of your scleroderma patients,” Sonja Bartolome, MD, a pulmonologist and associate director of the pulmonary hypertension program at the University of Texas Southwestern Medical Center, told attendees. “And for those that don’t have scleroderma — if they are dyspneic, think about and screen them.”
When treating patients with connective tissue diseases, rheumatologists should maintain high levels of suspicion of pulmonary arterial hypertension (PAH), Bartolome said, adding that early detection has the potential to dramatically alter and improve patient outcomes.
“The potential benefits of screening especially your scleroderma population — and, I would also argue, your dyspneic population who have other connective tissue diseases — are that the earlier we treat, the better they do,” she said. “We realized this really early in our clinical trials for pulmonary hypertension, because we are doing placebo controls.”
In early trials for pulmonary hypertension (PH) and PAH, patients who received placebo medication demonstrated noticeable differences in as short as 3 months, according to Bartolome. She added that patients who were then switched to placebo never did as well as patients who received medications from the beginning of the trial.
“We reiterated this on many of our clinical trials, and this is why you will not see placebo-controlled clinical trials for PH anymore in this country,” Bartolome said.
Screening for PAH is especially recommended for patients with scleroderma because of the prevalence of the disease in patients with scleroderma.
“Scleroderma is the most common thing by far that we see, both limited and diffuse, but more commonly limited,” Bartolome said. “Lupus would be second, mixed connective tissue disease third and rheumatoid arthritis last.”
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Bartolome. S. The role of the rheumatologist in the early diagnosis of pulmonary arterial hypertension in CTD patients. Presented at The Congress of Clinical Rheumatology West; Oct. 20-23, 2022 (hybrid meeting).
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