Lisa H. Lancaster, MD: Where the patient is in their disease, how sick they are, how impaired they are in their pulmonary physiology, and how much oxygen they may need certainly may guide your evaluation and assessment and discussions with the patient. There will be times we may consider 1 transplantation candidacy sooner rather than later for patients, and the same thing goes for palliative care and hospice discussions.
The 2 new therapies we have available that have been FDA approved for the treatment of pulmonary fibrosis are nintedanib and pirfenidone. They slow the disease progression by about 50%, but patients may not necessarily feel better. I think that’s a very critical and important point to get across to patients. Expectations are key to helping them maintain compliance and be able to take these medications for the long term.
We’ve been involved from the beginning at my academic medical center [Vanderbilt University Medical Center] with clinical trials with both nintedanib and pirfenidone and have followed patients who have been in clinical trials onto open-label studies and ultimately onto prescription therapy once the medications have been approved. We’ve had patients tolerate 1 better than the other and, if they couldn’t tolerate 1, switch and take the other 1 and tolerate it well in both directions. It’s really nice in this situation where prior to 2014, we had no therapeutic options but now have 2 therapeutic options and have a choice for patients.
Alicia M. Hinze, MD: This is really an exciting area of research that’s ongoing right now regarding the use of antifibrotic therapy for interstitial lung diseases in the context of autoimmune diseases. There are 2 antifibrotic therapies that are approved for the treatment of idiopathic pulmonary fibrosis. And there are ongoing studies to evaluate the efficacy of these antifibrotic treatments for the treatment of progressive-fibrosing lung disease in scleroderma. Recently there is an application to the FDA for a fast-track approval for nintedanib. So I’m really excited to see the phase 3 results of the trials that are currently underway for use of the antifibrotic therapies in the context of scleroderma related ILD [interstitial lung disease].
Lisa H. Lancaster, MD: We’ve learned a lot about treatment and our prior standard-of-care therapies in patients with idiopathic pulmonary fibrosis [IPF] since the first multicenter trials were started in IPF in the late 1990s. From the IPF Clinical Research Network PANTHER study, we learned that immunosuppressive medicines such as corticosteroids and Imuran are not helpful. In fact, they may be harmful for patients with idiopathic pulmonary fibrosis.
Alicia M. Hinze, MD: In scleroderma we don’t tend to see a lot of benefits with use of corticosteroids. Corticosteroids also have particular risks in the context of scleroderma, especially early on in the setting of a lot of skin involvement. Corticosteroids can increase the risk of something called scleroderma renal crisis. And because we don’t see a lot of necessarily long-term benefit with the use of corticosteroids, you really don’t tend to use them as much or at all. In patients, for example, with inflammatory myopathies with interstitial lung diseases, we do see some benefits, and we’ll often use them in the context of interstitial lung disease or really active muscle disease. In other conditions, perhaps if interstitial lung disease is underlying lupus, for example, then we may use steroids in that context too. From a rheumatology standpoint, the use of steroids really depends on what autoimmune condition is underlying the interstitial lung disease.