THE OFFICIAL NEWS SOURCE OF ACR CONVERGENCE 2022 • NOVEMBER 10-14



New IPF treatments may work for interstitial lung disease

New approaches to the treatment of idiopathic pulmonary fibrosis (IPF) may affect the treatment of interstitial lung disease in the setting of systemic sclerosis and other rheumatologic conditions.

New drugs for IPF could point the way to clinical trials in rheumatologic patients, while renewed focus on early and accurate diagnosis may help slow progression.

“Making an accurate diagnosis is not that difficult when patients have a clear-cut rheumatologic disease like rheumatoid arthritis or scleroderma,” said Paul Noble, MD, Pulmonologist and Chair of Medicine at Cedars-Sinai Medical Center in Los Angeles. “But there are a number of patients who present more with breathlessness and cough who have interstitial lung disease but have either an unclassifiable or mixed connective tissue disease or one of the antisynthetase syndromes who are more difficult to diagnose. Just a heightened awareness about the accurate diagnosis of a connective tissue disease when patients present with interstitial lung disease is important.”

Dr. Noble will explore emerging themes in IPF and their application to interstitial lung disease during a basic science symposium on Fibrosis and New Therapeutic Approaches on Monday. The most important clinical development was the approval of the first two drugs for any type of pulmonary fibrosis in 2014, pirfenidone and nintedanib.

Both agents were approved by the Food and Drug Administration for IPF, Dr. Noble said. Neither is indicated for use in interstitial lung disease. Off-label use is always a possibility, he said, although reimbursement for off-label use can be problematic.

“The primary endpoint for both of these agents was change in lung function as measured by forced vital capacity,” he said. “Both drugs reduced the rate of progression by about half over the course of a year. They don’t improve lung function, but they offer the hope that slowing progression could translate into a mortality benefit.”

Pivotal trials for the drugs were not powered to demonstrate any survival benefit, he said, nor were rheumatologic patients included in the two patient populations. Similarities between IPF and interstitial lung disease in rheumatologic conditions underline the need for trials in rheumatologic patients.