Chronic Fibrosing ILD: Presentation and Comorbidities

Multifaceted discussion on the presentation of chronic fibrosing interstitial lung disease with progressive phenotype and potential comorbidities.


Neil B. Minkoff, MD: Let’s get a slightly different perspective on this now and ask Ms Rapola to weigh in. You have chronic fibrosing ILD [interstitial lung disease] with progressive phenotype. I was wondering if you could tell us how your symptoms started and what your journey was to get to the diagnosis? How were you evaluated in the first place?

Dawn Repola: In 2008—2009, I started training for a half-marathon. I have been active all my life, and all of a sudden my times were getting slower. I decided I needed a coach, and my times were still slow. After a 4-mile run 1 morning, I nearly passed out. I decided I should go to the doctor. I went to see my PCP [primary care physician], and after a lung x-ray she thought I had pneumonia and put me on a course of antibiotics. It didn’t resolve, so she put me on another course of antibiotics. That didn’t help, so she sent me to an allergist. I am happy to report that the only thing I’m allergic to is tumbleweed. Then I saw a physician friend at dinner, and she was really worried because I was short of breath while eating. She said I need to see a pulmonologist. That’s when things started to change. My pulmonologist took a CT [computed tomography] scan of my lungs. I had blood clots in my lungs. I was fortunate that he also noticed that there was fibrosis. The blood clots had to be dealt with first, he said, and we have to watch this now because you have an insult to the lung. For 5 years I did pulmonary function tests every year.

Neil B. Minkoff, MD: To the clinicians I will ask, “Does this sound like a common story?” And can you comment on the comorbidities and complications associated with chronic fibrosis ILD that you’re looking out for when you see a patient like this?

Justin Michael Oldham, MD, MS: Yes. That is a pretty typical story that we hear. Patients unfortunately wait a long time from the onset of symptoms to get their ILD diagnosis. They unfortunately see a good number of doctors before that occurs. Unfortunately they are misdiagnosed a few times before they get to the correct diagnosis. This is a pretty classic story that we hear.

Laura Hummers, MD: I have to say, among the rheumatic disease, maybe we’re somewhat at an advantage or disadvantage depending on how you look at it. Since many of the other features of the rheumatic disease may present first, that gives us an opportunity to screen before somebody becomes symptomatic. If you have rheumatoid arthritis and you require medical attention because your joints are painful and swollen, and you have no breathing symptoms at all, it’s recognized that some patients with rheumatoid arthritis in scleroderma and some of the other rheumatic diseases have interstitial lung disease as a complication. Then we have the opportunity to screen and monitor for patients in an asymptomatic state and hopefully catch their progression sooner. But sometimes shortness of breath, even with the rheumatic disease, could be the presenting feature of their disease. Unfortunately that group of patients who present with lung disease first are more likely to be progressive just because they may not be symptomatic early on. By the time they present with shortness of breath, they may have already accumulated a fair amount of fibrosis.

Sonye Danoff, MD, PhD: To Dawn’s point, it would be great if everybody had just 1 thing, but that’s actually not the case. So for Dawn, she had the blood clots, the pulmonary emboli, as well as the interstitial lung disease. We see patients who present with other problems like pulmonary hypertension, coronary artery disease, or peripheral vascular disease. It would be nice if there was 1 thing, particularly with the progressive phenotype. [Are we] always looking for the progression of the interstitial lung disease, or is it something else? It’s easy to attribute everything to progression of the ILD when sometimes it’s exactly as Dawn says, it’s something entirely different, which may be easier to fix. As clinicians, a lot of what we do when we see patients who are not responding the way we had hoped is question if we are treating it the right way. Is there something else that’s happened that is modifying the patient scores? I’m sure Laura sees a tremendous amount of pulmonary hypertension in her scleroderma patients, and it can be very difficult initially to separate what’s progression of the interstitial lung disease versus what’s perhaps emergence or worsening pulmonary hypertension.

Laura Hummers, MD: Sometimes it’s both, just to make things more complicated. For our scleroderma population, I’ll just mention, almost all of them have gastroesophageal reflux disease, which is almost always the cause of cough. If people have a progressive or nagging cough, everybody is always worried that it’s their interstitial lung disease. But we have to keep some of these other features of the disease in mind, like reflux, which is a fairly common cause of cough.

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