How Is Chronic Fibrosing ILD With Progressive Phenotype Diagnosed?

Shared insight on the diagnosis of chronic fibrosing interstitial lung disease with progressive phenotype.


Neil B. Minkoff, MD: Dr Oldham, you mentioned that by the time many patients get to your practice or the practice of some specialists in this area, they’ve seen a number of other doctors and potentially been misdiagnosed more than once. What do you suggest to straighten that out, and how do you work through the process of getting to the correct diagnosis?

Justin Michael Oldham, MD, MS: When I see a patient, there’s usually an established rheumatologic diagnosis. Typically there’s interstitial lung disease. I don’t have to do much of that work, but a lot of patients still do come to me on inhalers with a diagnosis of COPD [chronic obstructive pulmonary disease] or asthma. Simply looking at the lung function tests and the CT scan helps you piece together that the patient doesn’t have much in the way of obstructive disease or significant emphysema. If they’re not noticing any benefit from the inhalers and they’re paying money for them, it makes sense to stop them. This is the quickest and most immediate change in their diagnoses and management that occurs when they see me. Most patients already know they have interstitial lung disease [ILD] by the time they get to me. Most of my focus is spent trying to categorize what interstitial lung disease is and how we manage it.

Neil B. Minkoff, MD: Dr Danoff, are you having the same experience in your practice? Or are you getting patients prior to their diagnosis of interstitial lung disease?

Sonye Danoff, MD, PhD: We see patients where the concerns about ILD have been raised. I work with the rheumatologists at [Johns Hopkins Medicine], and in our region we have patients who are sent to us where there is a new symptom of shortness of breath and there’s concern about defecting whether it’s related to interstitial lung disease or some other feature. We also see patients who have a CT but don’t have symptoms and get picked up with an asymptomatic or presymptomatic radiographic evidence of interstitial lung disease. We have a variety of different patients. Step 1 is to determine, is it an interstitial lung disease? We also see other things, we see people who come in with heart failure or people who have had some sort of an insult to the lung where they have a fixed scar in their lung and may not have an active interstitial lung disease or progressive interstitial lung disease. Trying to sort that out and also trying to identify what an appropriate therapy is. Obviously, there are many patients for whom we have very effective therapy. We can think about it like a person who has a hypersensitivity to a parrot in their home. If we can get the parrot out of the house and we can keep them away from bird products, they could recover perfectly well. We really watch the trajectory of symptoms and pulmonary function testing and so forth to get a better sense of what each person’s individual trajectory is.

Laura Hummers, MD: And to that point, sometimes we get referrals in rheumatology where somebody has had an interstitial lung disease identified and there is something about their history or their work-up so far that makes them think, “Oh, maybe they live in the rheumatic disease spectrum.” Which has important implications, from a prognosis standpoint for their lung disease. You also want to make sure you’re not missing some other part of their rheumatic disease and what we know about the rheumatic diseases are that if we can get somebody phenotyped, put them in the right box, and figure out which antibody they make, where they fit in on the spectrum of scleroderma or myositis or rheumatoid arthritis. It might tell us something about what other components of their disease we need to look for and how their interstitial lung disease might behave, if we can get them into the right box or phenotype them appropriately. Sometimes we’ll get referrals from our pulmonary colleagues and say, “Hey, you know, it seemed like they might fit in the rheumatic disease group. Where do they fit?” Sometimes people just don’t fit. And that’s pretty par for the course in rheumatology. There’s a lot of gray, but when we can, that can be helpful.

Neil B. Minkoff, MD: How do you communicate this to the patients? How do you help manage the trajectory of patient care? Is there a specific ICD-10 [International Classification of Diseases, 10th revision] code for chronic fibrosis ILD with progressive phenotype?

Justin Michael Oldham, MD, MS: I spend time on the provider side but also on the payer side, and there is not yet 1, although I understand that it is being developed. This leads to complexity on the payer side of trying to understand who is treating whom and for what. Is that something that’s challenging in your practices?

Sonye Danoff, MD, PhD: It is often challenging to communicate, through ICD-10 codes, what an individual’s trajectory is. I think both Laura and Justin have had this experience where we ask for a medication to be prescribed because the patient hasn’t tolerated another medication that would have been used before, and we’re asking for a higher cost or more complicated therapy. It’s hard to communicate that through just an ICD-10. Typically when we have an ICD-10 with the checklist that says, “Yes, I’ve tried this drug. Yes, I’ve tried that drug. No, they couldn’t tolerate this drug.” But we don’t have a good way of just saying this has been refractory to therapy or this has progressed even in the face of treatment with appropriate therapy. I was just dealing with this coding issue earlier today for a patient where I needed to ask for a medication for a prior authorization.

Laura Hummers, MD: In rheumatology it’s more complicated. I take care of scleroderma patients, so I’m kind of a lumber. I have 1 diagnosis in my chart for a patient that says they have systemic sclerosis. What I need to do to get medications and testing paid for is I need to say that they have systemic sclerosis. But then I separately need to code each disease they have—interstitial lung disease, pulmonary hypertension, reflux, Raynaud syndrome with ischemic digits—to get whatever drug I’m looking for to treat all those different manifestations of the disease. It’s a lumber or a splitter problem sometimes in rheumatology how you do the coding.

Justin Michael Oldham, MD, MS: I’ve found this particularly challenging on the rheumatologic side of things, trying to get more expensive immunosuppressive therapies. Now that we have 1 antifibrotic therapy, nintedanib, approved for progressive fibrosing ILD, it hasn’t been as big of an issue thus far. We’re only a few months into this process, but it is important to acknowledge that the patients who are studied in the trial identify nintedanib to be effective in these patients, who had documented progression prior to enrollment. Viewing this from a payer’s side, they certainly are going to want to know a patient is progressive before they approve an expensive drug like that.

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