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Approaching Interstitial Lung Disease Management


Alicia M. Hinze, MD: It can be very varied when patients seek care. Some people will not seek care until they actually develop symptoms of shortness of breath. They will perhaps go in to see their primary doctor for evaluation of shortness of breath and have either lung function studies or imaging, and it will show evidence that there is something abnormal with the lungs that is perhaps suggestive of interstitial lung disease. These patients will often, if they’re presenting with dyspnea, then go see a pulmonologist who will further evaluate the interstitial lung disease and potential drivers.

There’s another subset of patients I often see who will actually come in with rheumatic symptoms, so symptoms such as Raynaud phenomenon, joint pain and stiffness, or perhaps some other features of rashes or skin thickening. And based on the diagnosis of the actual autoimmune disease and my understanding of it, patients may be at particular risk for interstitial lung disease. I may then progress to obtaining additional work-up, such as a pulmonary function testing [PFT], to see if I am seeing any subclinical evidence of lung disease. Taking scleroderma, for example, there are certain subsets that are more at risk for interstitial lung disease. In subsets clinically or by laboratory, I do know that they are at higher risk for interstitial lung disease. I may go looking more intently for it in order to detect it, so we can better monitor it over time to determine need for treatment.

It is really a multidisciplinary effort in regard to treating and managing patients with interstitial lung disease. For patients, for example, who do have interstitial lung disease in the context of autoimmune disease, the pulmonologist and the rheumatologist are really working together very closely. The rheumatologist is also assessing some other features of autoimmune disease that may also require treatment, such as immunosuppression therapies. And so we are wanting to ensure not only that the patient’s lung disease is being treated and managed but also that we are addressing other features of the autoimmune disease that may also require immunosuppression. It really is a multidisciplinary care between rheumatology, as well as the pulmonologist.

There can be a pretty wide range in the severity of disease. There are patients, using scleroderma, for example, who may have a little bit of a radiographic interstitial lung disease. But these patients will have normal functional studies on PFT and will really not be particularly short of breath from interstitial lung disease, and they can remain in this category for their lives.

There’s another subset that may develop a few functional changes based on the interstitial lung disease in which they may become a little bit shorter of breath, and we can see some changes in the lung function or the PFTs themselves. And this is the group where sometimes we will monitor these patients to determine if they may progress. And if so, we will implement immunosuppressive therapies. There is another subset of patients that may progress more quickly, and instead of monitoring, we may implement therapies in a very quick fashion. It really is trying to assess where the patient is at, the level of symptoms that they’re having, and the timing of when it is best to implement therapies.

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