FDA-Approved Treatment Options for ILD

ILD treatment options approved by the FDA are explored by Dr Noble, MD.

Paul Noble, MD: The FDA has approved nintedanib and pirfenidone for the treatment of idiopathic pulmonary fibrosis [IPF], but there have been several additional studies with nintedanib in this progressive pulmonary fibrosis group. This was an interesting study in which patients who had pulmonary fibrosis from a number of causes were selected largely on the basis that they weren’t felt to have idiopathic pulmonary fibrosis: autoimmune related, chronic hypersensitivity, drug related, and some other less common forms of fibrotic lung disease. But to be included in the randomized placebo-controlled trial, they had to show evidence of disease progression. It had to be by either lung function, imaging, or symptoms.

This study demonstrated that if you were on nintedanib relative to placebo, you had less of a decline in your forced vital capacity [FVC]. It was very similar to the IPF study in the sense that patients didn’t improve. There was no improvement in exertional breathlessness, but the rate of decline of forced vital capacity was less. Similarly, nintedanib was also studied in scleroderma-related interstitial lung disease. Patients were randomized to receive nintedanib or placebo. Interestingly, about half the patients in that study were on immunosuppressive therapy: prednisone, mycophenolate, or some other immunosuppressive agent. It demonstrated that if you were on nintedanib, relative to placebo, you had a slower decline over 1 year in your forced vital capacity.

What was interesting was that the group that did the best in demonstrating the lowest amount of decline in FVC were patients on both immunosuppressive therapy and nintedanib. I find that intriguing because the general concept of using immunosuppressive therapy in an autoimmune disease is that the immune system is dysregulated. In essence, the body is trying to reject the lung. These immunosuppressive therapies target the underlying immune system, making it more difficult for the immune system to target the lung. It’s intriguing that the combination of immunosuppressive therapy with nintedanib showed the least amount of decline, suggesting that trying to target the underlying mechanism and the ability to reduce collagen formation was perhaps the best option in these patients.

More recently, there was an additional drug approved to treat scleroderma that was targeting interleukin-6. It was an interesting study in that the primary end point was negative, which was a softening of the skin or a change in the measures that rheumatologists use to demonstrate skin thickening in scleroderma. Although that primary end point was negative, there was a difference in the rate of decline of forced vital capacity. It would be useful to see more studies along those lines, because that placebo group behaved a bit differently from what many of us see in our patients with scleroderma. Nintedanib has expanded its FDA-approved repertoire to IPF, progressive pulmonary fibrosis, and scleroderma-related fibrosing lung disease.

Transcript edited for clarity.

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