New Insights into the Management of Interstitial Lung Disease

Research suggests patients with interstitial pulmonary fibrosis (IPF) do the worst while autoimmune diseases like SARD do the best. When an FVC decline of ≥10% was factored in, the transplant-free survival curves were the same across diseases. When other markers were looked at following removal of FVC decline, few were found to be prognostically useful.¹⁰

“What we find when we look at how well these measures predict death or transplant over the next year is that we are really good at identifying those who won’t die from PPF,” said Dr. Oldham. “But using current criteria, we are doing a bad job of finding those who will die.”

Biomarker Indicators

In an attempt to find better indicators, Dr. Oldham and others used machine learning to look for a composite biomarker. They completed a proof-of-concept study using a 12-protein signature. When applied to the validation cohort, the low-risk signature had a <10% chance of progression. The high-risk signature was more ambiguous.¹¹

“For a patient with a small chance of progressing, I would be comfortable using these biomarkers to monitor them over the next year,” he said. “We are working on finding additional proteins and moving it to a quantitive panel for use in the clinic.”

When Initial Management Doesn’t Work

Some studies guide initial management, but when things go wrong, PPF comes into play. It is important to think of PPF as a failure of traditional management.

Nintedanib has been the only option for treating PPF. Compared with placebo, nerandomilast in both 9 mg and 18 mg doses slowed FVC decline over time. There was also a survival benefit not only in the overall cohort, but in the autoimmune subgroup.¹²

“I think these data could easily change the way we treat this condition,” said Dr. Oldham. “This is a big step up over what we have been able to offer over the last six years.”

Disclosures

Dr. Wilfong is a consultant for Allogene, Capstan Therapeutics and AstraZeneca; an advisor, review panel member and consultant for Boehringer-Ingelheim; and a former advisor/review panel member for Merck/MSD.

Dr. Oldham is a consultant for GlaxoSmithKline and Roche, and an advisor, speaker or review panel member for Boehringer-Ingelheim.

Kurt Ullman is a freelance writer based in Indiana.

References

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