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Signs of Chronic Fibrosing ILD With Progressive Phenotype


Considerations for symptoms that are tied to chronic fibrosing interstitial lung disease with progressive phenotype.


Neil B. Minkoff, MD: Dr Danoff, you see a lot of these patients. Can you describe their symptoms?

Sonye Danoff, MD, PhD: The symptoms of progressive interstitial lung disease are very similar to the symptoms we associate with ILD [interstitial lung disease] in general. Those are the presence of shortness of breath, particularly with exercise or exertion; cough; and often a more diffuse sense of fatigue or weakness or inability to accomplish what the desired goal is. When we see a patient who has interstitial lung disease and we identify the etiology of the disease, we hope to make an intervention that stops the progression of the injury to the lung. [We hope] that their symptoms go along with that, that their symptoms improve as their lung disease improves. Unfortunately, even with appropriate identification of etiology and a preplan of appropriate treatment, there is a subset of patients who don’t respond in that way. As Justin nicely outlined, there are people who have progression in their lung function testing, in their CT [computed tomography] scanning, and that typically is associated with worsening symptomatology: becoming short of breath [more often], needing oxygen, needing more oxygen, being able to do less exertion, having a cough that becomes disabling. Many of those symptoms can go along with the progression of interstitial lung disease.

Neil B. Minkoff, MD: Does that go along with the range the other clinicians are seeing in terms of the severity of symptoms of the patients who come to them?

Laura Hummers, MD: Symptoms are variable from person to person, and that depends on what their baseline activity level is. Patients who are fairly inactive to start may not notice the early changes of lung fibrosis, because they’re not exerting themselves as much. Whereas for our patients who are very active, we’ll notice even small declines in their lung function. We have a group of patients who come in and feel their limitations as fatigue or generalized weakness or just inability to do things, which is fairly common. When you push them, they’ll say, “Yes, it is my breathing somewhat that’s limiting me,” but they really have a general sense of fatigue.

Justin Michael Oldham, MD, MS: That was nicely stated: Symptoms can range broadly. But as Sonye pointed out, a cough and shortness of breath seemed to be, at least in my anecdotal experience, the best metrics of progression.

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