All patients with systemic sclerosis require screening for interstitial lung disease, PAH
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SAN DIEGO — All patients with systemic sclerosis should be screened for pulmonary arterial hypertension and interstitial lung disease, according to a speaker at the 2024 Congress of Clinical Rheumatology West.
“This is a rare disease, and most rheumatologists only see about 10 or 12 scleroderma patients in a year,” Janet E. Pope, MD, MPH, FRCPC, professor of medicine at the University of Western Ontario, in London, Canada, told attendees. “So how do I predict who I’m supposed to worry about? When I go home at night, who do I worry about?”
According to what Pope called the “15% rule,” many of scleroderma’s severe complications affect approximately 15% of patients. That includes 15% of patients demonstrating clinically relevant ILD, and pulmonary arterial hypertension impacting approximately 8% to 15%, depending on the cohort, she said.
Pulmonary function tests (PFTs) and echocardiograms should be performed to check for pulmonary arterial hypertension, according to Pope. She also suggested asking patients whether they have felt increasingly short of breath when completing daily activities like walking with their partner, going uphill or rushing to answer a phone call.
“Ask about dyspnea compared to last year,” Pope said. “When you say, ‘Are you short of breath?’ they literally think you mean right now. And the answer is, ‘No, I’m not short of breath right now.’”
Pope stressed that diagnosis of pulmonary arterial hypertension requires a “right heart [catheterization] and needs an expert center to work with you.”
As for progressive ILD, studies have identified various biomarkers for which patients are at risk, such as anti-Scl-70, but many will still be negative, according to Pope.
“Especially early, listen for crackles,” she said. “Do PFTs more often early on, and later a bit less often.”
The biggest predictor of progressive ILD identified by Pope’s Canadian Scleroderma Research Group was what she called “barf lung.”
“If you wake up and you’re aspirating, you have barf in your mouth and you have needed an esophageal dilatation — those two things together — you have a high chance of progressing,” she said. “So don’t forget to treat the reflux aggressively.”
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Pope JE. Predicting Outcomes in Systemic Sclerosis by Skin Involvement and Autoantibodies. Presented at The Congress of Clinical Rheumatology West; Sept. 26-29, 2024. (hybrid meeting).
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