Lack of Guidance in Progressive Chronic Fibrosing ILD


Neil B. Minkoff, MD: Dr Oldham, as we start to think about treatment and the different treatment paradigms, what’s available clinically and available for patients? My understanding is there aren’t specific guidelines in ATS [American Thoracic Society] for chronic fibrosing ILD with progressive phenotype. And, what is the education that you are providing to your patients? And how are you making these treatment decisions independent of guidelines, which are often found in disease states like this?

Justin Michael Oldham, MD, MS: The field is in its infancy, as far as treating patients with chronic fibrosing ILD with the progressive phenotype. The INBUILD trial was just reported last October. It’s not surprising that we don’t have consensus guidelines from the major organizations. Until those come out, we are left relying on the clinical trial data. Which did show nintedanib was effective for slowing lung function decline in these patients compared to placebo. While we don’t have consensus guidelines, I rely heavily on the inclusion criteria for that study to guide my decision-making in patients who I think would meet criteria. That said, there are patients who have low to very low lung function that clearly had to progress to get there in the first place. We don’t have objective evidence with serial lung function or serial high-risk CT to show that progression. There is a case-by-case basis we need to make a call based on cross-sectional data, single time point. These patients probably are progressive; we just don’t have enough serial lung function or high res CTs to say so. But in general, if I do have serial lung function I do rely heavily on INBUILD inclusion criteria.

Neil B. Minkoff, MD: Do others use the inclusion/exclusion criteria in the same manner?

Laura Hummers, MD: I mostly take care of scleroderma patients and the census trial, which was the scleroderma trial for nintedanib had a more liberal inclusion. You didn’t have to have progression necessarily in that trial. And this is one of the biggest challenges we have in scleroderma is trying to figure out who that subset of patients are, that are going to progress. From the clinical trial data that we have in the past, that shows if you have advanced lung disease you’re probably going to continue to advance. Fibrosis is going to get more fibrosis. What we really need is some earlier biomarkers to tell us who at an early stage is going to be in that group of patients, who has fibrosis, but it’s never going to cause them trouble. Versus those who are going to continue to progress before they get to the point where it’s obvious they’re going to continue to progress. That’s one of our biggest challenges is identifying that subgroup of patients who will progress, who are going to be the candidates for therapy.

Sonye Danoff, MD, PhD: Just extending from what Laura was saying, you can think of it as being analogous to cardiology. And, years and years ago we waited till the person had their first heart attack, and then we figured out what we needed to do to keep them from having their 2nd, 3rd and 4th heart attacks. Now what we’re thinking about is more primary prevention, how do you prevent? How do you risk stratify a person? And then prevent that very first heart attack. And that’s where we are in interstitial lung disease. We recognize when patients have the interstitial lung disease. We can monitor them progressing, having their 2nd, 3rd, or 4th heart attack, having their acute exacerbation. What we want to do is have those biomarkers that will let us say before they develop any symptoms or when they had the most minimal symptoms. This is somebody where we really need to focus our attention to prevent progression. And that’s sort of the Holy Grail for a lot of disease states. But for sure it’s the Holy Grail for lung disease. Whether you come at it from the perspective of being somebody who takes care of autoimmune interstitial lung disease, or somebody who sees patients who are being treated for another disease state and get a drug, and that drug causes interstitial lung disease. We all want to minimize the risk to our patients and maximize their lung function. Which we know tends to be associated with maximizing their life expectancy as well.

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