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Unmet Needs and Future Directions in ILD


Alicia M. Hinze, MD: Some of the unmet needs that we have in interstitial lung disease [ILD] in the context of scleroderma would be, what do we do if patients continue to progress on our immunosuppression therapies or our first-line therapies? There is this concept, perhaps early on in the disease course of interstitial lung disease, that it may be very immune driven or inflammatory driven and that immunosuppression drugs may be the best. But as time goes on, or as the process goes on, there is also a thought that it turns into more of a profibrotic phenotype that can be self-sustaining based on our knowledge of other fibrotic lung diseases. Presently we don’t have therapies that are really targeting that profibrotic process that is thought to occur.

In the scleroderma community, we are all excited to see the results of the 2 studies that are ongoing that are looking at these antifibrotic therapies and whether we’re going to be able to see some benefits with these antifibrotic lung therapies in the treatment of ILD associated with scleroderma.

Lisa H. Lancaster, MD: For patients with idiopathic pulmonary fibrosis or other interstitial lung disease who are experiencing hypoxia, we need to develop better delivery systems for oxygen. We also need to develop better payment systems for oxygen so that patients can get the equipment they need. We need further development of smaller systems that deliver higher flow rates as patients with interstitial lung disease with time and with disease progression require higher flow rates of oxygen than somebody with emphysema or certain other lung diseases.

This is an exciting time to take care of patients with IPF, idiopathic pulmonary fibrosis, and interstitial lung disease because there are many clinical trials that are evolving and have evolved over time looking at any fibrotic therapies that abrogate various pathways of the fibrotic process in the lungs. We have a lot of opportunities to learn from these clinical trials. Even if they’re negative, we learn a lot about the disease process, and we learn a lot about the different pathways that get us ultimately to those fibrotic pathways, typical interstitial pneumonitis, and NSIP [nonspecific interstitial pneumonia].

Gary L. Johnson, MD, MBA: Looking ahead to the future, in the last several years we’ve had 2 expensive drugs that are utilized to treat lung fibrosis. I’m sure there are many more that are coming along in the pipeline. The mechanism of action of 1 of those drugs is still not entirely clear, but it is approved and is something that will provide a base for future research and development by the pharmaceutical companies. The costs associated with treatment will clearly be rising, but if we can get benefit from those drugs, it’s something that we as an insurer have an obligation to pay for.

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