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Interstitial Lung Disease: Immunosuppressive and Nonpharmacological Options

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Alicia M. Hinze, MD: In regard to treatment options, I’ll speak from the rheumatic side of treating interstitial lung disease, using scleroderma as probably 1 of the prototypical rheumatic diseases in which we see interstitial lung disease. The therapies that we presently use are immunosuppressant drugs, specifically mycophenolate mofetil. There have been some studies, 2 particular studies of interstitial lung disease and systemic sclerosis, that we base our management on. The initial study is called the Scleroderma Lung Study 1, evaluated cyclophosphamide versus placebo and did find benefit in regard to reduction in the progression of interstitial lung disease when evaluating cyclophosphamide versus placebo, for example.

The second study compared mycophenolate with cyclophosphamide and determined that there is really no difference between those medications. The conclusion is that mycophenolate really is as effective as cyclophosphamide, which has previously been shown to be of benefit when compared with no drugs at all. Mycophenolate has a better toxicity profile, so that really is our standard when we decide that a patient does need to go on immunosuppressant therapy. Mycophenolate, for example, would be our first line in scleroderma.

Within the rheumatic diseases, other immunosuppressive treatment options for interstitial lung disease are guided somewhat by the actual rheumatic diseases underlying the interstitial lung disease. For example, we also see interstitial lung disease in the context of inflammatory myopathy. We’ve found that in these conditions we were able to use other drugs, such as azathioprine or Imuran or rituximab. And so these will be medications that we can also employ in the treatment. Looking at scleroderma, mycophenolate is really our standard medication that we’ll use first line for a treatment.

Some of the lifestyle habits, first and foremost, would be stopping smoking if that is something that patients are doing. Other things would be management of gastroesophageal reflux disease. It usually is a combination of medications but also lifestyle changes, which can include reducing caffeine or reducing carbonated beverages, trying to eat smaller meals throughout the day instead of a few large meals, and trying to separate the timing of meals to bedtime to try to reduce those symptoms. Other modalities we’ll use in interstitial lung disease will be pulmonary rehabilitation. This has been shown in studies to affect walking distance, for example, and health-related quality of life. Some of those are nonpharmacological modalities for managing that can at least improve patients’ symptoms and can be associated with improvements and 1 of our parameters of monitoring, which can be the 6-minute walk test.


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