Isabelle Amigues, MD, on the Need for a Switch in Terminology: From CTD-ILD to AI-ILD

In a recent tweet, Isabelle Amigues, MD, posed the following question, “Is the world ready to stop using CTD-ILD and use rheumatologic related autoimmune disorder (AI-ILD) instead? As a rheumatologist it pains me every time to use this outdated denomination.” The tweet spurred much conversation among her peers in rheumatology. In this podcast, Dr Amigues expands on the topic. She explains the reasoning behind why she and other rheumatologists suggest that practitioners stop using the term “connective tissue disease-associated interstitial lung disease” and how doing so can benefit patients.

Podcast Transcript:

COLLEEN MURPHY: Hello, everyone. Welcome to another installment of the Autoimmune Learning Network’s podcast series. I’m your moderator, Colleen Murphy, with the Autoimmune Learning Network.

Today I am joined by Dr Isabelle Amigues. Dr Amigues is a rheumatologist at National Jewish Health in Denver, Colorado, where she also serves as an assistant professor in the Division of Rheumatology and Department of Medicine.

I was on Twitter recently when I came across one of Dr Amigue's tweets. She had tweeted, “Is the world ready to stop using CTD-ILD, and use rheumatologic-related autoimmune disorder, AI-ILD, instead? As a rheumatologist, it pains me every time to use this outdated denomination.”

Now, Dr Amigues, after seeing this tweet and the reaction that your followers had to it, I really wanted to get more of your thoughts on the topic. I’m excited to be able to talk with you about it. Thanks for joining me.

ISABELLE AMIGUES: Thank you very much for having me.

CM: Let’s get into why you think there’s a need for change to stop using the term connective tissue disease-associated interstitial lung disease.

IA: I think that if you talk to any of us rheumatologists you’d see that we consider connective tissue disorder only real connective tissue disorder, meaning Henderson nodes, Marfan syndrome, these things that are more genetic and are related to an abnormal function of the tissue.

For example, in Henderson nodes, you have very thin skin, thin vessels sometimes. That is the main issue of their problem. They will have scars. They will have hyperlaxity. They will have some type of aortic aneurysm. When we are talking about connective tissue disorder in rheumatology, we are not talking about this anymore. We are still using the term that’s outdated, connective tissue disorder, when we use it in an association with interstitial lung disease. We all understand autoimmune disorder. If we all understand autoimmune disorder, why can't we use that? That’s my question.

When I talk to rheumatologists, all of us are like, “No, we should use autoimmune disorder and not connective tissue disorder.” That came out to this. I was writing a note. I was [laughs] again writing CTD-ILD patient referred to rule out CTD-ILD. I was like, “This is not CTD.” [laughs] It just came from there.

CM: I want to expand on that. Over the years, using this label of CTD-ILD, how has it affected practice and specifically, maybe even the treatment of patients? Does it have a negative effect at all using that term?

IA: I don’t think it does. Here’s the thing. When we talk to all rheumatologists and pulmonary specialist that specialize in interstitial lung disease, we all have the same word chocie. We are talking about autoimmune-related interstitial lung disease, scleroderma-related ILD, rheumatoid-arthritis-related ILD, antisynthetase syndrome or dermatomyositis, including myositis-related ILD. So I don’t think, from a physician perspective, it is so much of an issue in terms of managing the patients. 

The first issue is for the patients. When they hear that term, they don’t know what we’re talking about. When you say it’s autoimmune, then suddenly, it’s much clearer what’s happening. If you say, “You have a connective tissue disorder,” they are like, “OK, why are you giving me an immunosuppressive agent?” [laughs] If you say that, “It’s autoimmune. It’s your immune system attacking your own,” then they understand why we have to use agent that immunosuppress them. That’s the first thing.

The second thing is that the same way when we take care of cancer patients — I do not — but when oncologists take care of patients, they have done such a tremendous job. They are very precise. We’re not talking about breast cancer; we’re talking about a specific type of breast cancer. No one would be OK with just saying, “He has cancer.” You have to tell which type of cancer, which organ and, in which organ, what type of specific cancer they have.

In interstitial lung disease, we have to also do this. We are doing it. It’s just that this broad spectrum of CTD personally bothers me and bothers some of my patients. They’re like, “There’s nothing CTD about me. It’s just autoimmune.” A lot of us are wanting to change that term. It’s just this old baggage that we’re carrying around with us.

With all the advances that have happened in your past 20, 10 years about interstitial lung disease and rheumatologic disease, it’s time now to change and to say, “OK, well, we know that connective tissue disorder-associated interstitial lung diseases are actually autoimmune.” If they are not just autoimmune, at least they are closer to autoimmune than they are to CTD.

To talk about this a little bit more, there was a consortium of specialists in interstitial lung disease—rheumatologists, pulmonary, radiologists, and pathologists—that tried to come up with a term that would encompass this idea. That’s called the IPAF, or interstitial pneumonia with autoimmune features.

The issue that I have with it is that, 1, it’s actually more complicated than it sounds. It’s basically ruling out like when you have another rheumatologic disorder, it’s not IPAF. IPAF is when you don’t know. When I’ve tried to use it in practice, I find it difficult. We are always wondering in our ILD conference, multidisciplinary conference, “Oh, is this IPAF?” Then we realized it doesn’t make any difference. We are trying to get there. It’s just we’re not there yet.

Finally, the other thing that is important is insurances are giving us trouble. For example, we are trying to say, “OK, this patient has CTD-ILD;” you will not get the medications that you necessarily want. If we were to say, all of us, “This is autoimmune interstitial lung disease,” we may have access to a little bit more offered by insurance. They’re covered by insurances. 

CM: That’s an interesting point. You touched on a couple of points that I have questions about coming up. That’s great. One of them was you were talking about cancer and treating the specific types of cancer. That made me think about one of the comments under your tweet.

A lot of doctors were coming on and were explaining how they choose to group the diseases. One doctor tweeted that, when applicable, he uses IPAF. Otherwise, his practice tries to call it by what disease or pattern it is associated with, such as RA ILD-UIP.

Another doctor tweeted that her practice group usese CTD as more SLE/Sjogren/scleroderma. The other 2 groupings that she has would be for inflammatory arthritis, RA/PsA, and then spondyloarthritis. What else are your peers saying about the change?

Like I mentioned earlier, this tweet got a lot of traction on Twitter. Many of your peers agreed with you that there is a need for change. One of them on Twitter even saying that CTD is outdated and confusing. I’m interested in knowing what else your peers are saying too, whether it’s on Twitter or outside of Twitter. Any pushback or suggestions?

IA: No. This is very [laughs] interesting to me. That’s where I’m not understanding this. All the rheumatologists that I know, they all agree that this is autoimmune-related ILD.

When anyone sends up the patient saying rule out CTD- ILD, we all understand and know that is rule out autoimmune-related ILD. I don’t know any rheumatologist that does not want to change that name. It’s going to take more time and/or more energy to try to change the name. And the truth is that the only other word, and you did see this with IPAF. Someone came up with IPAF, but IPAF is outdated. IPAF is too specific. We need something that is more generalized to explain this process that we don’t know of, same analogy with cancer.

In cancer, you have an idea of what it means, but then you have specific cancer, specific organ, specific presentation. That’s the same thing. It’s autoimmune, and then you have specific presentation. Clearly, we don’t treat scleroderma ILD the same way that we treat rheumatoid arthritis ILD or antisynthetase syndrome.

That’s where it’s very funny for me to read, is that every single rheumatologist that I know is OK with that “autoimmune.” I think the pushback is more from pulmonary because for them, they called it CTD forever. For them, it doesn’t make a huge difference. At least, it doesn’t sound like it’s as much of an issue for them than it is for us rheumatologists. Again, it’s not a huge issue. It’s just, to me, something that makes me cringe every time I write it.

CM: You also tweeted that even though all rheumatologists agree on it, if you were to use AI-ILD in a paper or grant proposal, that it would be rejected. And like you said, other communities have been able to make such changes.

You point out that the renal community has been able to adopt AKI vs ARI. So if rheumatologists are all agreeing on this, is it frustrating that there is still this kind of force that doesn’t really allow you to go ahead with the change?

IA: First of all, I remember when ARI became AKI. And it wasn’t easy, but I would say that they all came together and it was OK. But it was only one type of specialty; it was nephrology. In this case, we are talking about 2 specialties. We are talking about pulmonary and rheumatology.

I’m sure rheumatologists would come together and be OK with saying, “OK, we’re just going to call it AI.” But it also has to be approved by the pulmonary community as well. Maybe someone is going to write a paper and explain why we need to change this. I don’t recall, but I’m sure it was coming from a paper that said, “Let’s stop using renal failure and use kidney failure instead.” Maybe someone very bright and very renowned in the ILD world changes to AI-ILD, and we can finally move on. [laughs] It won’t change the fate of the word, but make some rheumatologists cringe a little bit less.

CM: You know what, I guess this is another example of how challenging a multidisciplinary approach can be.

IA: Yeah, that’s a good point. The other point is that we get along so well. In National Jewish, we consider we are part of this 1 team, ILD and rheumatologists. And so it’s not worth the fight.

I remember presenting a grand proposal, I had changed all CTD-ILD to AI-ILD. My mentor was like, “It’s going to make people cringe. They’re just going to be like, ‘Why are you changing something that has been used forever?’”

I was like, “Yeah, actually, that is true. I’m new in this word of ILD. I’m not going to change the word by changing this on my proposal.” I just went back to put everything CTD-ILD. Sometimes I think that’s what it is; we are just feeling that it’s not worth to fight over it. It’s very surprising that the renal...It’s cool to see that the...I wonder how they were able to do it, the nephrology team, where the nephrologists were able to change from using renal and use now kidney. It was pretty fast, but it had to come from...Everyone has to be willing to do that. The truth is that it’s so ingrained. And when your mentors are telling you, “Well, the reviewer is going to read this and they are going to be like, ‘What is this? Why does she want to change this denomination?’” Then you just end up using the same old, same old. And that’s OK; you are still doing the job.

CM: Wow, this has all been interesting. If any of our listeners are interested in giving you a follow on Twitter to see what other thoughts you have, at what handle can they find you?

IA: Absolutely. Yeah. It will be my pleasure. It’s @isa75012. It’s a former zip code where I used to live in Paris when I did my medical school, 75012. [laughs]

CM: I was wondering what those numbers were for. That’s interesting.

IA: [laughs] That’s my attachment to Paris medical school. That’s where I found that I wanted to be a rheumatologist.

CM: Dr Amigues, I want to thank you again so much for your time. It’s been really interesting.

IA: Thank you. Thank you for having me and for starting the conversation. It’s an interesting conversation.

Isabelle Amigues, MD, is a rheumatologist at National Jewish Health in Denver, Colorado. There, she also serves as an assistant professor in the Department of Medicine, Division of Rheumatology.

Follow Dr Amigues on Twitter (@isa75012).

Additional Resource:

@isa75012. Is the world ready to stop using CTD-ILD and use Rheumatologic related autoimmune disorder (AI-ILD) instead? As a rheumatologist it pains me every time to use this outdated denomination. @leticiakawano @jeffsparks @RheumILD. November 17, 2020 Accessed December 15, 2020. https://twitter.com/isa75012/status/1328781416025395202

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