‘Be aggressive’ in detecting, managing interstitial lung disease in early scleroderma
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DESTIN, Fla. — Detection and treatment of interstitial lung disease in systemic sclerosis calls for early and aggressive action, according to data presented at the Congress of Clinical Rheumatology East.
“Most of us really try to be more conservative in scleroderma,” Dinesh Khanna, MD, MSc, a professor of medicine at the University of Michigan Medical Cener, told attendees. “Be aggressive in this patient population.”
The preferred methods to screen for interstitial lung disease (ILD) are high-resolution CT and a pulmonary function test (PFT), while invalid methods include chest radiograph and hall walk distance, according to Khanna. He added that all patients with early scleroderma should be screened for ILD.
“Screening at this time is critical because if you wait for them to have symptoms of ILD, they probably have lost 40% to 50% of their lung volume,” Khanna said.
He added that the dose of radiation from high-resolution CT is “equal to what you get on an annual basis.” However, if radiation or other concerns call for more targeted screening, providers should focus on patients with the diffuse cutaneous subtype of scleroderma. Khanna additionally urged rheumatologists to screen patients who are positive for anti-SCL-70 antibodies, also called anti-topoisomerase antibodies, which he called a key risk factor for ILD.
“If you have a patient with symptoms of systemic sclerosis and anti-SCL-70, they are at a very high risk fir developing progressive ILD,” Khanna said.
He added that anti-centromere antibodies act as a protective factor, conferring an ILD risk of “about 10%.”
However, it is shortly after the diagnosis that rheumatologists usually make the “biggest blunder” in treating ILD, according to Khanna.
“They diagnose somebody with ILD, they have mild ILD, and then they say, ‘OK, we’ll see you back in a year or 2,’” he said. “If you have taken the responsibility of diagnosing somebody with ILD, you have to do a close follow-up. The recommendations are pulmonary function tests every 3 to 6 months until you show stabilization, along with ambulatory desaturation testing at least once a year.”
The first-line treatment for most patients in Khanna’s clinic “continues to be mycophenolate,” he said. For progressive disease, steroids are contraindicated, while potential options include immunosuppressives, nintedanib (Ofev, Boehringer Ingelheim) and autologous stem cell transplant, he added.
Khanna emphasized that treatment for ILD is “long-term.”
“I’ve made many mistakes, and one of them is I used to stop mycophenolate,” he said. “If you stop the drug, the risk for flare is about 20% to 30%. My motto is that if patients are doing fine on 2 grams, 1.5 grams a day of mycophenolate, just leave them on.”
He added that non-melanoma skin cancers are “the thing that you have to worry about” with mycophenolate.
“They’re very common,” Khanna said, adding that patients “have to see a dermatologist once a year in my practice.”
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Khanna D. New guidelines in the diagnosis and management of SSc-ILDs: How to incorporate them in practice? Presented at The Congress of Clinical Rheumatology East; May 9-12, 2024; Destin, Florida (hybrid meeting).
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