Unpacking CTD-ILD Controversies

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Good evening.

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Good evening, everybody,
and I'm so glad we've got a great

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attendance for this Master class series
in Rheumatology and ILD.

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So first of all,
some easy things to tell everybody.

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You will be muted till the end and so
your microphones will be off and cameras

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will be off.

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But at the end you can come on camera and
you can ask a question throughout.

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The questions are in chat.

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If it's a burning question,
I'll try to interrupt the speakers in a

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very polite way and get it answered
because I think interactivity in real

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time sometimes is more helpful.

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So I'm Janet Pope,
I'm a rheumatologist and professor of

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medicine at Western Schulich School of
Medicine and Dentistry in London, ON.

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We have two very superb speakers,
which is a real pleasure to introduce.

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So for those of you in the rheumatology
side, who is most of you.

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Many of you already know Doctor Sandra
Chartrand.

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She is a fellow of the Royal College,
Physician of course, and rheumatologist.

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She's at the hospital of
Maisonneuve-Rosemont in Montreal, Quebec.

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She's an assistant clinical professor in
the Department of Medicine at U of M.

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She completed her training in internal
medicine and rheumatology there as well

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and had her fellowship in autoimmune and
pulmonary diseases in Colorado in Denver,

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Colorado.

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She's an excellent speaker,
huge insights and she has a real focus on

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connective tissue disease,
especially lung involvement such as ILD,

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and she has an active interdisciplinary
ILD and autoimmune lung disease clinic.

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She has a diverse practice spanning ,
really, tertiary care, community,

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rheumatology and she does outreach as
well.

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So it's a real pleasure to have her.

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Many of you also know Professor Martin
Kolb.

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I'm not sure if everyone does yet.

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He is a guru in rheumatology.

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He's at McMaster,
He's a professor and holds a Chair.

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So it's the Gauldie BI Chair in ILD and
Research Director at the Firestone

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Institute for McMaster University.

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for ILD.

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His research is focusing for many years
on fibrotic lung disease and he looks at

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the basic science aspects,
so growth factors, matrix, etcetera,

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pulmonary vascular remodeling as well.

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He has been part of multiple ILD trials
and also has been on DSMB and

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adjudication boards for it.

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He has multiple peer reviewed papers and
his H factor is extremely high, 88, that,

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for those who don't know what that means,
that's like ginormously high 30's,

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pretty good, 88's kind of outstanding.

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So that's great.

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He's been on the editorial board and an
editor of multiple journals,

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and it's a real pleasure to have them
both here today.

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If we look at some other housekeeping,
as I mentioned, as people were coming in,

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cameras will be off till the end.

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Ask any questions in real time in the
chat box.

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We'll try to have time for questions at
the end.

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You will be muted,
but if you think you're not muted,

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you can mute it yourself.

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There will be an eval at the end and it's
really important because BI really values

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the evaluations to help design the best
Masterclass for the Rheumatologist.

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If we move on to financial support,
I think it's interesting and important to

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note that this program has had financial
support from BI and some logistical

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support from them and their team as well
for potential conflict of interest.

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Really,
there's a mitigation of bias where at all

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possible,
but if you feel there's a conflict,

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we would be glad to hear about it.

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Probably not necessarily in real time,
but afterwards,

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but I don't think you're going to feel
that that way as you hear the important

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data that will be discussed.

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The other thing is if we move on,
they would to basically the disclosures

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will be given.

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And I think it's really important again
to note that the content was developed by

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the speakers and that BI had no input.

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They just made the slides pretty and made
them look nice and formatting.

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If we move on to the objectives,
we have about 3 objectives that I think

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we'll be able to accomplish tonight.

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Describing key information for
respirologists and rheumatologists.

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So exchanging and coordinating ILD care
using examples through cases on

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interpreting the PFTs and giving HRCT
trends of interpretation and obviously

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other clinical features of ILD and really
talking about collaboration of

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therapeutics and the decision making,
who's responsible for what in other words

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and who's going to follow the patient
when.

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And that's really important for our
patients with CTD-ILD including RA,

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Scleroderma, etc.

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So with that,
I will hand it over for the the next

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slide and we'll get going.

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All right, so lovely.

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Thanks Janet for this kind introduction
and it's a delight to be here.

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Talk to the rheumatologist and I've seen
a few respirologist as well on the list

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here.

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But I am very confident with talking to
rheumatologist because I work very

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closely in my clinic with,
with a couple of them.

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So I,
I would just want to set the stage a

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little bit and just provide a few reasons
why you as rheumatologist should really

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care somewhat about interstitial lung
disease.

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And there are four different things I
wanted to, to just like remember.

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So I work in a speciality clinic for
interstitial lung disease,

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and in the past pretty much everything
that people thought we do is IPF.

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So idiopathic pulmonary fibrosis,
really a bad disease,

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no cause found and it has a pretty bad
mortality rate.

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But in fact,
when we look at what we're doing in these

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clinics,
an equal number of patients all have

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actually some kind of rheumatological
disease.

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And I already used the word of SARD here
that I think this is a newly coined term

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from the rheumatologist and the in the
group here.

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So as a pulmonologist,
we often still refer to CTD-ILD,

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so connective tissue disease.

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But of course we acknowledge that not
every rheumatological disease is

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connective tissue disease.

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So I just adapt this systemic autoimmune
rheumatological disease ILD now.

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But anyway,
it's about 20% of what I do in my

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practice.

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So it's important,
we see these patients quite regularly.

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Second point is that a lot of these
diseases that we call SARD or CTD-ILD or

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like IPAF or idiopathic pneumonia with
autoimmune features,

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which is not clearly defined,
but all that group that you would sum up

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under the SARD-ILD is a big contributor
to what we call progressive pulmonary

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fibrosis.

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So not all the diseases that are SARD-ILD
would lead to progressive pulmonary

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fibrosis,
but a good number would do so and a good

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portion of these progressive pulmonary
fibrosis diseases is- has an underlying

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SARD component.

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Now this is quite important because what
we call as a respirologist.

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PPF,
progressive pulmonary fibrosis is

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essentially a group of diseases that is
behaving like IPF,

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idiopathic pulmonary fibrosis,
but it has a different underlying reason

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for it.

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And behaving like - means people progress
and develop symptoms and die with pretty

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much the same speed as for IPF.

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This PPF definition has been really
established and changed in the last

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couple of years.

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But from a respirology perspective,
it's actually relatively simple.

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So we call it PPF: progressive pulmonary
fibrosis.

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When a patient has worsening symptoms,
has physiological evidence of progression

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or has radiological evidence of
progression,

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if it have two out of those three in the
past year,

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then we call it progressive pulmonary
fibrosis.

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And Martin, just a quick question.

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You had said a slide before that they
progress about the same rate as IPF.

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I think one thing we tend to think in the
rheumatology world is that our SARD-ILD

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patients,
they do progress if they've already got

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poor, poor prognosis features.

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But we always kind of think they take
longer to succumb,

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to die than the than the IPF.

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Am I wrong in thinking that?

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And you are right,
and you're wrong because of course all

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the SARD-ILDs are not the same, right?

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So, so and it is,
it is really a big factor of determining

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progression and the speed of progression
is the image pattern.

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Also,
if you have a pattern which we call usual

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interstitial pneumonia,
that's the same pattern we see in IPF.

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And if you have that pattern and your
underlying SARD is rheumatoid arthritis,

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you progress pretty much at the same
speed.

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If you're underlying disease is Sjogren's
,

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you progress pretty much at the same
speed.

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Yeah.

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So there's some determinants of
progression.

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Yeah, but some are definitely better.

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Yeah.

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So some of the SARD-ILDs are clearly
better than than IPF.

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OK.

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But that's - so,
so absolutely a good point.

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And I,
I'll actually get back to that in a

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little bit with a different perspective.

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Yeah.

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So just to move back,
so physiology means we just measure how

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big are the lungs.

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So we measure lung volume,
forced vital capacity.

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It's essentially how deep a breath can
you take and how much air can you take in.

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Smaller lungs means lower numbers.

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And that's for clinical research mostly
our primary endpoint.

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And we also measure the gas transfer,
which you are very familiar with because

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you know since many years that this is
one of the things you should do as a

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screening for all the patients diagnosed
at least with Scleroderma.

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So that measures,
measures the gas transfer.

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Radiology is probably a little bit of a
black box for rheumatologists when it's

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regarding the lungs because you don't
look at these pictures.

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If you feel bad when you look at the
picture,

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I can reassure you that probably 3/4 of
respirologists feel equally bad when they

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look at these pictures because it often
needs quite a specialized eye and

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experience to,
to look at these images with the same

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competence as a radiologist would have.

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But the, the, the document here,
if you're interested in really just

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highlights what, what do we look at?

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So what has get worse of these different
components here?

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But it's, it's pretty well defined and,
and we know what to look for.

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The symptoms of these diseases that are
called PPF are the same as for IPF.

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The ways how we assess these patients
from respirology is the same.

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We measure lung function, lung volume,
we measure how far can someone walk in a

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6 minute walk test.

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We look at quality of life,
we monitor for acute exacerbation.

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So there's really nothing different
between how we manage a patient with IPF

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versus PPF,
except of course the interventions that

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we have, the pharmacology.

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Third reason is the one that I find most
important: patients with IPF or PPF.

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And as I said before,
a lot of these PPF patients have SARD-ILD,

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they die at a rate that is worse than
most cancers that you know of.

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There's only two cancers that are worse
in terms of five year survival,

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which is lung cancer and pancreatic
cancer.

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But all the other cancers that affect our
patients and people in Canada and all the

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developed world are not as deadly as lung
fibrosis.

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And that's a very important thing.

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It is basically at the moment when you as
a physician have someone diagnosed with

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IPF or PPF,
you tell them your chances of dying in

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five years is 50% and that's pretty
serious.

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Yeah.

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And this is where you should not wait,
you should take steps in order to prevent

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that.

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And that's my 4th point here now,
because the SARD-ILDs are in terms of

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what we can do with drug intervention,
pharmacological treatment,

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definitely better than IPF.

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So conceptually,
a patient with IPF always progresses and

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the best you can do with pharmacological
intervention is you stop the progression

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or you slow it down.

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On average, we slow it down.

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In contrast,
many of the diseases that are SARD-ILD,

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with differences between them can be
improved with the therapy that you know

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much better than I,
since you use them all the time in your

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practice.

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And that really brings me just for a few
minutes of talking about inflammation and

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fibrosis.

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So inflammation and fibrosis in
interstitial lung disease is really

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affecting the treatment decisions that we
have.

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And just keep in mind,
the reason why an individual would get

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fibrosis or also rheumatoid arthritis is
usually including a genetic

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predisposition and an external or
internal insult.

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That could be pollution and smoke,
could be lifestyle,

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could be autoimmunity and so forth.

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Yeah,
but you have a predisposed individual

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that has an exposure through their
lifetimes and that leads to disease.

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And when we look at the patient in the
ILD clinic,

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really have to ask these questions.

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So what is the likelihood of stable
disease without doing anything?

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What is the likelihood of having -dealing
with an inflammatory disease that would

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trigger the use of anti-inflammatory
treatment or what is the likelihood of

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progressive fibrotic disease that would
trigger antifibrotic therapy?

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And there are a few clinical criteria
that we in respirology use in order to

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determine that.

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So if someone had immunosuppressants,
often a steroid trial is how the

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respirologist call it without any effect.

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It's not likely to,
to respond to anything.

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If there are different patterns that that
you see like the usual interstitial

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pneumonia pattern on the right side here.

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If you have certain demographic factors
like older men for instance,

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or have often progressive fibrosis that
is less responsive.

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Younger women have more autoimmune
disease so this is more commonly

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associated with treatment response to
anti-inflammatory drugs.

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We look at lavage with lymphocytes being
present.

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Again,
this speaks more for the benefit of

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immunosuppression.

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Neutrophils are not responding to
anything.

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Was tried numerous times,
just doesn't work.

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But those are all factors that we can
take into account when we make a

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treatment decision.

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Can we ask one question again, Martin?

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So we in general,
our patients don't get lavage unless if

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something is very different because BAL
for us,

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we say if we're going to treat the
underlying disease anyway,

00:16:21.132 --> 00:16:23.120
we're hoping to help the lungs.

00:16:23.360 --> 00:16:27.216
If they're progressing,
they'll probably be eligible for PPF kind

00:16:27.216 --> 00:16:28.560
of approved treatments.

00:16:29.840 --> 00:16:35.044
So should we look at BAL or should it be
only in a case that is actually

00:16:35.044 --> 00:16:40.320
extraordinary where something's not
fitting in our SARD kind of patients?

00:16:40.560 --> 00:16:44.040
Yeah, I, I think it's,
it's probably in something is not fitting.

00:16:44.040 --> 00:16:50.730
You would think about it more interesting
enough the the use of BAL in making

00:16:50.730 --> 00:16:52.360
treatment decisions

00:16:52.960 --> 00:16:58.000
There are very, there are,
there are profound geographic differences.

00:16:58.440 --> 00:17:01.200
So in the United States,
they don't like BAL

00:17:01.840 --> 00:17:03.960
In Europe,
it's done in anyone who has ILD.

00:17:04.440 --> 00:17:07.920
Also in Japan. Canada is like in between.

00:17:07.920 --> 00:17:09.080
Some do it, some don't.

00:17:09.680 --> 00:17:13.796
Yeah, but as a respirologist,
when you have a very typical image,

00:17:13.796 --> 00:17:15.480
you don't do a BAL anymore.

00:17:15.680 --> 00:17:18.440
There's a,
it's an older man with a UIP pattern.

00:17:19.440 --> 00:17:20.480
I don't do it right.

00:17:20.480 --> 00:17:23.767
But if it's a younger woman with a mixed
pattern,

00:17:23.767 --> 00:17:28.895
I do it because it gives me some some
reassurance that the the process in the

00:17:28.895 --> 00:17:32.840
lungs is driven by enrichment of
lymphocytes, for instance.

00:17:32.840 --> 00:17:33.000
Yeah.

00:17:33.000 --> 00:17:35.920
So but that's that's exactly how it's
also included in the guidelines.

00:17:35.920 --> 00:17:38.000
It's it's it's more optional.

00:17:38.640 --> 00:17:38.920
Yeah.

00:17:38.920 --> 00:17:45.840
And people use that optional tool very
variably between - with no good reason.

00:17:46.200 --> 00:17:47.280
is it really does.

00:17:47.280 --> 00:17:49.520
It's it's just probably what you used to
do.

00:17:50.920 --> 00:17:56.069
Yeah, but but a good point,
and if you just think biology,

00:17:56.069 --> 00:18:00.520
nothing of what I talk about is
intrinsically bad.

00:18:00.760 --> 00:18:00.960
Yeah.

00:18:00.960 --> 00:18:06.193
So we need to have inflammation in order
to survive and defend ourselves against

00:18:06.193 --> 00:18:06.840
intruders.

00:18:07.360 --> 00:18:08.800
We have to have resolution.

00:18:09.280 --> 00:18:13.240
And if one of these doesn't stop,
then you have a problem.

00:18:13.440 --> 00:18:13.640
Yeah.

00:18:13.640 --> 00:18:18.480
But the the the mechanism in itself is
not a bad mechanism.

00:18:18.480 --> 00:18:20.000
Just when it doesn't stop.

00:18:20.000 --> 00:18:24.960
And this is where where often the cause
of these diseases is.

00:18:26.160 --> 00:18:33.041
My really favorite example of this,
this flow from inflammation to fibrosis

00:18:33.041 --> 00:18:34.400
is Scleroderma-

00:18:34.400 --> 00:18:39.814
ILD and Janet will be probably very
easily detecting this pattern on this

00:18:39.814 --> 00:18:40.400
picture.

00:18:40.400 --> 00:18:43.850
Yeah,
but this is a woman like mid 40s who I

00:18:43.850 --> 00:18:45.920
followed over 7 or 8 years.

00:18:45.920 --> 00:18:46.600
Those pictures.

00:18:46.600 --> 00:18:46.720
Yeah.

00:18:46.720 --> 00:18:51.340
And you see literally on the left side
where you see a ground glass dominant

00:18:51.340 --> 00:18:52.840
radiographic abnormality.

00:18:52.840 --> 00:18:55.440
This is what we call nonspecific
interstitial pneumonia.

00:18:55.440 --> 00:19:00.600
You see that Halo around the pleural
space, so that's sparing. In the middle,

00:19:00.600 --> 00:19:04.029
a couple of years later,
you'll see how these ground glass areas

00:19:04.029 --> 00:19:05.560
get a little bit more coarse.

00:19:05.560 --> 00:19:08.320
So, of course means more,
more thick white lines.

00:19:08.840 --> 00:19:12.315
And on the right side,
you basically see almost only white lines

00:19:12.315 --> 00:19:14.240
and you see traction bronchiectasis.

00:19:14.240 --> 00:19:14.400
Yeah.

00:19:14.400 --> 00:19:19.911
So this woman had clearly an autoimmune
process that was floridly active on the

00:19:19.911 --> 00:19:20.600
left side.

00:19:20.600 --> 00:19:24.040
And then over the years she developed
pulmonary fibrosis.

00:19:24.040 --> 00:19:24.160
Yeah.

00:19:24.160 --> 00:19:30.080
So because there are and there's no clear
cut transition in the middle.

00:19:30.200 --> 00:19:30.360
Yeah.

00:19:30.360 --> 00:19:33.080
So that's another important point to to
think about.

00:19:33.080 --> 00:19:36.361
And and people who pay for drugs,
for instance,

00:19:36.361 --> 00:19:40.600
they say you treat either inflammation or
you treat fibrosis.

00:19:40.600 --> 00:19:41.520
You can't have both.

00:19:41.880 --> 00:19:43.800
They have changed slowly.

00:19:44.320 --> 00:19:46.200
Yeah, But in the beginning,
that's what they said.

00:19:46.240 --> 00:19:49.160
And that's just like,
that doesn't make sense, right?

00:19:49.160 --> 00:19:53.000
Because these processes, of course,
are are transiting into each other.

00:19:53.480 --> 00:19:53.800
OK.

00:19:54.760 --> 00:20:00.040
And that's that's what a respirologist
would would do in the clinic.

00:20:00.040 --> 00:20:02.240
So if we see a patient with ILD.

00:20:03.200 --> 00:20:07.477
We really try to see if that patient is
having something that is idiopathic in

00:20:07.477 --> 00:20:10.077
the middle,
then we know it's only antifibrotic

00:20:10.077 --> 00:20:11.160
therapies that help.

00:20:11.920 --> 00:20:16.103
If someone has exposure driven disease
like fibrotic hypersensitivity

00:20:16.103 --> 00:20:18.853
pneumonitis,
you would put a lot of effort in

00:20:18.853 --> 00:20:22.199
identifying the reason for it because you
can avoid it.

00:20:22.200 --> 00:20:23.200
Then you do better.

00:20:23.440 --> 00:20:26.320
If it's ongoing inflammation,
you think about immunosuppression.

00:20:26.960 --> 00:20:28.840
And I use CTD-ILD here.

00:20:28.840 --> 00:20:32.360
So sorry Janet,
it's SARD-ILD now on the right side.

00:20:32.360 --> 00:20:35.840
This is when we need the rheumatologist
here to to get the input.

00:20:35.840 --> 00:20:44.090
And this brings me now really to give
this microphone to Doctor Chartrand and

00:20:44.090 --> 00:20:46.840
she can get the let's see.

00:20:46.840 --> 00:20:47.920
Hopefully this works all.

00:20:48.000 --> 00:20:49.920
So Sandra, you should have control now.

00:20:50.720 --> 00:20:51.960
Yes, it seems to work.

00:20:52.360 --> 00:20:58.984
So thank you very much Martin for setting
the stage as we as rheumatologists we

00:20:58.984 --> 00:21:02.958
have to learn all these new terms and all
this,

00:21:02.958 --> 00:21:08.920
this new - I would say ensemble of
disease that can affect our patient.

00:21:09.320 --> 00:21:09.840
Yes.

00:21:09.960 --> 00:21:14.796
So in rheumatology we,
we have many different scenarios in which

00:21:14.796 --> 00:21:18.666
we can see,
we can see patient and we can encounter

00:21:18.666 --> 00:21:20.080
patient having ILD.

00:21:20.440 --> 00:21:25.049
So first it's the patient you have as a
rheumatologist and is known for a

00:21:25.049 --> 00:21:28.040
connective tissue disease that is well
defined.

00:21:28.240 --> 00:21:31.774
And at some point you're wondering if the
patient is at risk of developing

00:21:31.774 --> 00:21:33.000
interstitial lung disease.

00:21:33.440 --> 00:21:37.014
On the other side,
you have your patient that you - is known

00:21:37.014 --> 00:21:41.937
for any connective tissue disease that is
developing ILD during the follow up under

00:21:41.937 --> 00:21:45.160
treatment you give for that connective
tissue disease.

00:21:45.400 --> 00:21:50.591
And also more and more we see patients
referred by pulmonologists asking if this

00:21:50.591 --> 00:21:55.846
patient has a possible connective tissue
disease to explain the interstitial lung

00:21:55.846 --> 00:21:57.000
disease they have.

00:21:57.080 --> 00:22:01.664
So as Doctor Kolb said,
20% of all ILD and some places it may be

00:22:01.664 --> 00:22:03.640
a little bit more than that.

00:22:04.080 --> 00:22:10.915
These the patient having interstitial
lung disease have CTD or rheumatologic

00:22:10.915 --> 00:22:14.200
disease when we look for it properly.

00:22:18.080 --> 00:22:20.200
I'm trying to change a slide.

00:22:20.400 --> 00:22:23.240
So let's give, let's see an example.

00:22:23.240 --> 00:22:27.634
So for the purpose Miss RA,
she's a woman of 44 years old and she

00:22:27.634 --> 00:22:32.693
presents to you with joint pains,
mostly hands and wrists for the past four

00:22:32.693 --> 00:22:33.160
months.

00:22:33.600 --> 00:22:37.697
She presents with swelling mostly in the
morning with prolonged stiffness at the

00:22:37.697 --> 00:22:38.760
on the physical exam.

00:22:38.760 --> 00:22:43.320
She has multiple synovitis of MCP and PIP
and also of the wrist.

00:22:43.680 --> 00:22:45.200
She has elevated CRP.

00:22:45.200 --> 00:22:50.257
She has a positive rheumatic factor and
anti-CCP and you will see no erosions at

00:22:50.257 --> 00:22:52.880
the beginning on the hand X-rays for sure.

00:22:52.880 --> 00:22:55.040
This is what we see most days.

00:22:55.120 --> 00:22:57.920
It's a diagnosis, it's simple,
it's rheumatic arthritis.

00:22:58.200 --> 00:23:02.257
At this stage,
you have to ask yourself is this patient

00:23:02.257 --> 00:23:05.880
already have ILD or is she at risk of
having ILD?

00:23:08.000 --> 00:23:11.684
So we have to remember that the
prevalence of interstitial lung disease

00:23:11.684 --> 00:23:15.470
in connective tissue disease is quite
high in different connective tissue

00:23:15.470 --> 00:23:15.879
disease.

00:23:15.880 --> 00:23:21.108
For sure RA is not the highest but since
the the the number of patient having

00:23:21.108 --> 00:23:26.537
rheumatoid arthritis - 10% is quite high
for sure systemic sclerosis is the most

00:23:26.537 --> 00:23:31.498
often I would say interstitial lung
disease patient in CTD that we see as

00:23:31.498 --> 00:23:35.520
rheumatologists,
but their incidence is a little bit lower.

00:23:35.520 --> 00:23:39.609
But you see that all the connective
tissue disease we have - most of them

00:23:39.609 --> 00:23:43.920
they have quite a number of patient that
can have ILD during their follow up.

00:23:44.160 --> 00:23:49.277
And I think as rheumatologists it explain
why we have to ask ourselves if our

00:23:49.277 --> 00:23:54.461
patient are having ILD at the moment we
counter them and ask every time we see

00:23:54.461 --> 00:23:59.120
them if they develop interstitial lung
disease during their follow up.

00:23:59.720 --> 00:24:04.965
Also we have to remember that having
interstitial lung disease in patient with

00:24:04.965 --> 00:24:09.878
connective tissue disease is highly
associated with higher mortality than

00:24:09.878 --> 00:24:12.800
other presentations or other
manifestation.

00:24:13.040 --> 00:24:16.120
And it's not the same for every
connective tissue disease.

00:24:16.320 --> 00:24:20.090
For sure we see a lot of interstitial
lung disease in Scleroderma,

00:24:20.090 --> 00:24:22.960
but their overall mortality is a little
bit lower.

00:24:23.200 --> 00:24:28.407
In rheumatoid arthritis,
it may be higher probably because this

00:24:28.407 --> 00:24:34.673
patient have more UIP patterns and they
are kind of - the evolution is quite

00:24:34.673 --> 00:24:37.440
significantly the the same as IPF.

00:24:37.600 --> 00:24:43.009
But you say we can also see that in
patient having inflammatory myositis

00:24:43.009 --> 00:24:47.160
usually because they present more acute
severe disease.

00:24:47.160 --> 00:24:50.008
So the,
the mortality is mostly at the beginning

00:24:50.008 --> 00:24:50.880
of the disease.

00:24:50.880 --> 00:24:56.819
So we have to state - so patient having
ILD in our patients,

00:24:56.819 --> 00:25:00.520
they can impact their, their survival.

00:25:01.000 --> 00:25:04.273
Sandra,
just one thing I think as well that the

00:25:04.273 --> 00:25:08.093
RA patients, the,
the mortality is high because they're

00:25:08.093 --> 00:25:11.640
often older,
either older onset or longstanding RA.

00:25:11.680 --> 00:25:16.038
So they're a bit older than,
say the mean disease that we see in our

00:25:16.038 --> 00:25:20.776
the mean age in our RA patients,
but also men and there's a lot of men who

00:25:20.776 --> 00:25:22.040
smoke in that group.

00:25:22.360 --> 00:25:26.825
So they probably have all those other
factors that are poor prognosis even in

00:25:26.825 --> 00:25:28.600
UIP patients who don't have RA.

00:25:29.320 --> 00:25:30.200
Yeah, for sure.

00:25:30.400 --> 00:25:34.120
So they share common,
I would say risk factor as IPF patients

00:25:34.120 --> 00:25:34.960
to start with.

00:25:34.960 --> 00:25:40.778
And we know now they'd have genetic and
also like I would say pathophysiology

00:25:40.778 --> 00:25:43.240
that is similar in a lot of ways.

00:25:44.080 --> 00:25:46.931
So when we see this patient,
first we see it's a,

00:25:46.931 --> 00:25:49.840
it's a woman of middle age presenting
with new RA.

00:25:50.200 --> 00:25:51.440
Would it make a difference,

00:25:51.440 --> 00:25:56.614
If you think about is she at risk of
having ILD if the patient was a 28 years

00:25:56.614 --> 00:26:00.063
old woman,
if the patient was an active smoker with

00:26:00.063 --> 00:26:04.840
a 20 pack per year smoking history,
would the patient - would it make a

00:26:04.840 --> 00:26:10.280
difference if it was a 68 years old man
and if the RA was diagnosed 11 years ago?

00:26:10.480 --> 00:26:15.240
So all these are good question because
probably it impacts the risk.

00:26:15.960 --> 00:26:20.230
So first I would remember everyone that
we have to go back to basics - as

00:26:20.230 --> 00:26:23.578
rheumatologists,
we have to document all the condition of

00:26:23.578 --> 00:26:27.213
the patient that could explain any kind
of dyspnea in patient,

00:26:27.213 --> 00:26:31.600
because all dyspnea are not necessarily
signs of interstitial lung disease.

00:26:31.600 --> 00:26:34.240
And we have to remember about the other
disease they have.

00:26:34.760 --> 00:26:39.324
Also we should take,
we should question respiratory symptoms a

00:26:39.324 --> 00:26:41.280
little bit more frequently.

00:26:41.440 --> 00:26:45.320
So we have to ask them on top of their
joint symptoms.

00:26:45.480 --> 00:26:47.240
Do you have an unexplained dry cough?

00:26:47.280 --> 00:26:51.120
Do you - have you lost some exercise
tolerance in the past few months?

00:26:51.960 --> 00:26:56.726
Are you limiting your exercise because
you're out of breath really easily and

00:26:56.726 --> 00:27:00.760
you cannot improve it while trying to
walk more or exercise more?

00:27:01.080 --> 00:27:05.056
And also,
do you have to slow your friends or your

00:27:05.056 --> 00:27:05.680
husband?

00:27:05.800 --> 00:27:08.800
Does he have to slow down when you walk
together?

00:27:09.440 --> 00:27:12.059
So,
and we can even ask if they are out of

00:27:12.059 --> 00:27:16.934
breath while talking, dressing, washing,
because sometimes it's way more severe

00:27:16.934 --> 00:27:18.640
than it shows in our office.

00:27:19.160 --> 00:27:24.080
Also, just the way, an easy way just to,
to, to see them come to your office.

00:27:24.160 --> 00:27:27.020
Know that the,
if the patient is dismayed because when

00:27:27.020 --> 00:27:30.972
they are sitting down in your office or
usually they are not out of breath,

00:27:30.972 --> 00:27:34.300
but sometimes just seeing them come up
and come to your office,

00:27:34.300 --> 00:27:37.680
you can see they are out of breath way
more than when they talk.

00:27:39.560 --> 00:27:45.480
I will just go back, my slide changed,
but I didn't change it.

00:27:45.800 --> 00:27:48.840
So otherwise, bring back your stethoscope.

00:27:49.160 --> 00:27:54.200
Auscultate the patient's lungs because
sometimes you have the sign with yourself.

00:27:54.200 --> 00:27:55.320
It's already there.

00:27:55.320 --> 00:27:58.543
You, you are,
you hear that the lung is like fibrotic

00:27:58.543 --> 00:28:03.080
and when they, they, they, they, they,
they breathe and it's already there.

00:28:03.080 --> 00:28:05.720
And also a trick that we have here in
Quebec,

00:28:05.720 --> 00:28:09.280
we have access to all the imaging they
have done in the past.

00:28:09.280 --> 00:28:15.385
It's in what we call the DSQ and we can
see sometimes that it's already somewhere

00:28:15.385 --> 00:28:16.800
in another imaging.

00:28:16.800 --> 00:28:22.000
So sometimes people have a scan of the
abdomen few years ago for other reason.

00:28:22.160 --> 00:28:27.200
And the the radiologist is already
describing ILD at the base of the lungs.

00:28:27.200 --> 00:28:31.880
And it's a chance for us because most of
our disease, they start at the base.

00:28:31.880 --> 00:28:35.206
So it's a good idea to just go through
all these images,

00:28:35.206 --> 00:28:39.467
these report that sometimes shows you,
you don't even ask to have to ask

00:28:39.467 --> 00:28:41.160
yourself, it's already there.

00:28:43.120 --> 00:28:48.171
It's so important that now we have
guidelines that are for rheumatologists

00:28:48.171 --> 00:28:51.000
to look for ILD in our patient population.

00:28:51.080 --> 00:28:57.258
So the ACR and the chest,
they came in 2023 and they kind of rename

00:28:57.258 --> 00:28:58.440
our diseases.

00:28:58.440 --> 00:29:02.280
So now it's systemic autoimmune rheumatic
disease instead of CTD-ILD.

00:29:02.360 --> 00:29:05.520
But it's just a matter of terminology.

00:29:05.520 --> 00:29:11.651
We want to include all patient in
rheumatology that may have interstitial

00:29:11.651 --> 00:29:15.960
lung disease,
but they mostly focus on CTD-ILD, so,

00:29:15.960 --> 00:29:19.440
so they have recommendation for screening.

00:29:19.440 --> 00:29:23.240
So they say all patient who have like a
SARD of interest.

00:29:23.240 --> 00:29:26.120
So mostly all our CTD patient.

00:29:26.480 --> 00:29:29.172
You have to ask yourself as a
rheumatologist,

00:29:29.172 --> 00:29:31.280
do they have increased risk for ILD?

00:29:31.600 --> 00:29:34.249
If you consider that they don't have any
risk,

00:29:34.249 --> 00:29:36.560
maybe just wait for the symptoms to come.

00:29:36.840 --> 00:29:41.482
But otherwise you should question
yourself if they are at risk and they

00:29:41.482 --> 00:29:45.029
suggest different,
I would say risk factor in patient,

00:29:45.029 --> 00:29:49.929
in patient with different connective
tissue disease in which maybe if these

00:29:49.929 --> 00:29:53.153
factors,
these risk factor are there depending it

00:29:53.153 --> 00:29:57.344
could be clinical manifestation,
it could be certain antibodies,

00:29:57.344 --> 00:30:00.440
it could be certain like epidemiologic
factors.

00:30:00.640 --> 00:30:04.640
Then you should at the beginning of a new
CTD diagnosis,

00:30:04.640 --> 00:30:08.080
maybe go and look for interstitial lung
disease.

00:30:08.080 --> 00:30:13.395
Even though the patient may not be short
out of breath, dyspneic or having cough,

00:30:13.395 --> 00:30:18.321
the difficulty with these risk factor,
they don't really help us to to say,

00:30:18.321 --> 00:30:23.378
do you - do we have to have all these
risk factor or just one is enough or is

00:30:23.378 --> 00:30:24.480
it a combination?

00:30:24.640 --> 00:30:26.480
So there's a little bit of feeling there.

00:30:26.800 --> 00:30:31.993
And unfortunately there's there are
evidence that if you if you look at those

00:30:31.993 --> 00:30:37.320
risk factor, you can miss unfortunately,
patient who are at risk or having ILD.

00:30:37.680 --> 00:30:43.054
So this study was done for the systemic
sclerosis patient and they say even if

00:30:43.054 --> 00:30:48.360
you have like, like no risk factor,
you will not catch 25% of those patients.

00:30:48.360 --> 00:30:52.116
So it's kind of scary to know that even
though you look at you're,

00:30:52.116 --> 00:30:55.480
you're following the recommendation,
you may miss patients.

00:30:55.640 --> 00:31:00.582
But that's why systemic sclerosis
patients should be screened all the way

00:31:00.582 --> 00:31:02.520
because they are all at risk.

00:31:07.240 --> 00:31:07.960
Otherwise.

00:31:07.960 --> 00:31:09.280
How do you screen?

00:31:09.280 --> 00:31:14.680
If you you determine that the patient is
at risk, then what are you doing?

00:31:14.720 --> 00:31:17.240
Is it chest X-rays are not enough?

00:31:17.600 --> 00:31:20.160
Just like auscultate the lungs,
it's not enough.

00:31:20.160 --> 00:31:23.000
Just asking the patient if he's out of
breath is not enough.

00:31:23.120 --> 00:31:28.852
So unfortunately we have to do the real
investigation we do to like catch the

00:31:28.852 --> 00:31:29.440
disease.

00:31:29.440 --> 00:31:31.720
And it relies on two things.

00:31:31.920 --> 00:31:37.677
First proving that there is ILD with high
resolution chest CT scans and then you

00:31:37.677 --> 00:31:43.080
want to know how they are affected by the
disease already at the beginning.

00:31:43.080 --> 00:31:45.160
So you have to have pulmonary function
test.

00:31:45.160 --> 00:31:49.828
This is kind of your baseline so it can
help you if the ILD is just mild,

00:31:49.828 --> 00:31:54.560
you know where to start and you'll be
able to catch progression over time.

00:31:54.960 --> 00:32:00.079
So unfortunately all the other - like
they don't recommend, as I said,

00:32:00.079 --> 00:32:05.560
chest radiography or 6 minute walk test
or just desaturation on ambulation.

00:32:05.560 --> 00:32:08.040
Bronchoscopy,
surgical lung biopsy for sure.

00:32:08.080 --> 00:32:11.776
We don't do that for screening,
but you really have to have your scan to

00:32:11.776 --> 00:32:13.600
make the diagnosis at the beginning.

00:32:14.920 --> 00:32:22.188
There are also other recommendations that
came out in the 2025 from the ERS/EULAR

00:32:22.188 --> 00:32:22.720
guide.

00:32:22.840 --> 00:32:25.560
The guidelines - they are quite different.

00:32:25.560 --> 00:32:31.097
So they just said it depends on the CTD
of interest how you should screen these

00:32:31.097 --> 00:32:31.720
patients.

00:32:31.960 --> 00:32:36.861
They recommend that systemic sclerosis
patients and MCTD patients should be

00:32:36.861 --> 00:32:38.280
screened, all of them.

00:32:38.600 --> 00:32:43.049
Then when we look at the different ones,
they say look for the risk factor and if

00:32:43.049 --> 00:32:45.600
there are risk factor,
you should screen them.

00:32:45.600 --> 00:32:49.840
If there are no risk factor,
there are less evidence to screen them.

00:32:49.840 --> 00:32:53.112
But they recognize that in inflammatory
myositis,

00:32:53.112 --> 00:32:55.600
these patients are quite high in risk.

00:32:55.600 --> 00:32:58.603
So maybe even without risk factor,
you should look for it,

00:32:58.603 --> 00:33:00.640
but the recommendation is not as strong.

00:33:01.040 --> 00:33:06.480
And for RA and Sjogren's, they say, well,
probably you should rely on risk factor.

00:33:06.680 --> 00:33:09.200
And they suggest a different risk factor
for patients.

00:33:09.200 --> 00:33:13.120
But we have the same problem because is
it just one risk factor?

00:33:13.120 --> 00:33:17.560
So is it just being older instead of just
being like younger?

00:33:17.560 --> 00:33:20.480
Is it being a man or a woman?

00:33:20.480 --> 00:33:24.800
Is it like the duration of the disease,
certain antibodies?

00:33:24.800 --> 00:33:26.240
Is it a combination of that?

00:33:26.480 --> 00:33:31.022
It- unfortunately these are all like
subject to research to come because we

00:33:31.022 --> 00:33:35.924
don't have the perfect combination or the
calculator to tell you: don't screen or

00:33:35.924 --> 00:33:37.119
screen this patient.

00:33:39.920 --> 00:33:42.832
I'm sorry,
I have problem looking for changing my

00:33:42.832 --> 00:33:43.240
slides.

00:33:43.680 --> 00:33:46.480
OK, so if we go back to Miss RA.

00:33:46.720 --> 00:33:49.200
So you judge that she has enough risk
factors.

00:33:49.200 --> 00:33:50.160
She's a little bit old.

00:33:50.160 --> 00:33:52.600
She she's been whatever.

00:33:52.600 --> 00:33:57.556
So you decide that you go for a pulmonary
function test and a chest CD scan and

00:33:57.556 --> 00:34:00.840
you're - she's happy,
no signs of ILD to start with.

00:34:00.840 --> 00:34:03.680
She have normal pulmonary function test.

00:34:03.840 --> 00:34:05.760
The problem is it's the beginning.

00:34:05.800 --> 00:34:10.177
You will follow that patient for many
years and you know that even though she

00:34:10.177 --> 00:34:13.040
doesn't have ILD to start with,
she has that risk.

00:34:13.040 --> 00:34:16.160
She has a disease that can make ILD come.

00:34:16.160 --> 00:34:19.280
So do you continue to look for the ILD
through years?

00:34:19.280 --> 00:34:24.000
And if yes, which test do you use?

00:34:24.000 --> 00:34:26.720
Is it okay not to do the scans anymore?

00:34:27.120 --> 00:34:31.640
Are you only relying on symptoms or do
you use pulmonary function tests?

00:34:32.360 --> 00:34:37.374
So if we look at the ACR recommendation
once you started to - even if the patient

00:34:37.374 --> 00:34:40.676
doesn't have interstitial lung disease to
start with,

00:34:40.676 --> 00:34:45.080
you consider they were high risk so you
should continue to follow them.

00:34:45.080 --> 00:34:50.267
And probably they say consider yearly
rescreening for high risk patients and if

00:34:50.267 --> 00:34:53.640
you decide to do it then they have
recommendations.

00:34:53.640 --> 00:34:57.355
So if there is ILD,
then they recommend like repeating

00:34:57.355 --> 00:35:02.353
pulmonary function tests and HRCT scan
through different like I would say

00:35:02.353 --> 00:35:07.149
intervals through the first years and
thereafter if you don't find any

00:35:07.149 --> 00:35:07.960
progression.

00:35:08.160 --> 00:35:13.008
But still even without ILD,
they recommend to continue to follow

00:35:13.008 --> 00:35:13.680
patients.

00:35:13.680 --> 00:35:15.480
ERS - they don't really talk about that.

00:35:15.960 --> 00:35:17.880
So when we go back to Ms. RA again.

00:35:17.880 --> 00:35:22.760
So you started her on like usual care,
methotrexate and hydroxychloroquine.

00:35:22.960 --> 00:35:25.640
At first she was well controlled,
but she had a few flares.

00:35:25.640 --> 00:35:30.537
At at some point you decided to add a
biologic - adalimumab - and then she

00:35:30.537 --> 00:35:32.040
developed few erosions.

00:35:32.040 --> 00:35:36.043
Few years after that,
you see that few deformities where where

00:35:36.043 --> 00:35:40.300
like starting when you, you,
you started your biologic and then at

00:35:40.300 --> 00:35:45.320
some point you continue your, you know,
your screening, but nothing was there.

00:35:45.320 --> 00:35:49.480
And then after a few years she starts
complaining she's, she's out of breath.

00:35:49.480 --> 00:35:52.280
So you ask her the question,
are you out of breath?

00:35:52.280 --> 00:35:55.240
Do you have the same exercise tolerance,
etc.

00:35:55.240 --> 00:35:57.728
And at some point she says, "well,
it changed, Doctor,

00:35:57.728 --> 00:35:59.720
since the previous time a few months ago.
"

00:35:59.960 --> 00:36:02.080
And you decide, well, we have to go back.

00:36:02.080 --> 00:36:03.960
Even though it's been many years.

00:36:04.240 --> 00:36:08.280
We go back to CT scans and pulmonary
function tests.

00:36:08.280 --> 00:36:12.140
And unfortunately,
you see that there are some reticulation

00:36:12.140 --> 00:36:17.029
with ground glass opacity and it's
described by the radiologist as moderate

00:36:17.029 --> 00:36:17.480
extent.

00:36:17.640 --> 00:36:24.160
And she already lost a lot of capacity -
forced vital capacity unfortunately.

00:36:24.360 --> 00:36:29.581
And this is something that happens a lot
because some of our patients are not I

00:36:29.581 --> 00:36:34.738
would say like really active and they
don't really like feel the out of breath

00:36:34.738 --> 00:36:36.240
until it's really late.

00:36:36.960 --> 00:36:38.440
So this is unfortunate.

00:36:39.120 --> 00:36:41.040
So it's the time to ask help

00:36:41.080 --> 00:36:45.894
This is where the collaboration starts
because the first part we cannot send all

00:36:45.894 --> 00:36:49.520
our patient with connective tissue
disease to pulmonologist.

00:36:49.520 --> 00:36:53.640
They will be have way too, too,
too much job to do.

00:36:53.760 --> 00:36:58.501
So at some point would have as
rheumatologist integrate the screening of

00:36:58.501 --> 00:37:03.632
our patient and the follow up when the
situation is there is no interstitial -

00:37:03.632 --> 00:37:06.880
ILD for now,
but there may be some at some point.

00:37:07.520 --> 00:37:10.820
And then when you have the proof that
it's there,

00:37:10.820 --> 00:37:14.320
it's time to ask collaboration with a
pulmonologist.

00:37:14.480 --> 00:37:17.761
So the patient,
when the patient has symptoms or there's

00:37:17.761 --> 00:37:21.272
proof of progression for sure,
you have to ask your favorite

00:37:21.272 --> 00:37:23.000
pulmonologist to get involved.

00:37:23.600 --> 00:37:27.684
Also, even without symptoms,
once you see there's ILD or if it was

00:37:27.684 --> 00:37:30.732
really mild and pulmonary function tests
were OK,

00:37:30.732 --> 00:37:33.475
but you followed up and there's
progression,

00:37:33.475 --> 00:37:38.352
you have to ask a pulmonologist to to
take back because there's progression and

00:37:38.352 --> 00:37:40.120
there are treatment for that.

00:37:40.440 --> 00:37:44.367
So as rheumatologists,
it's not our role to assume the diagnosis

00:37:44.367 --> 00:37:46.120
or the cause of ILD for sure.

00:37:46.280 --> 00:37:50.992
There are many other causes that have
been - have to be investigated before

00:37:50.992 --> 00:37:53.720
saying this is the CTD that caused the
ILD.

00:37:54.080 --> 00:37:56.840
So our patient are under
immunosuppression.

00:37:56.840 --> 00:38:00.040
Sometimes they develop weird pulmonary
infections.

00:38:00.320 --> 00:38:04.720
Sometimes they get other medication that
can cause a pulmonary toxicity.

00:38:04.720 --> 00:38:07.560
So these we are not really familiar with.

00:38:07.560 --> 00:38:10.840
So our pulmonologist will know where to
catch that.

00:38:11.040 --> 00:38:18.160
Sometime they have exposition to a lot of
different allergens, organic or inorganic.

00:38:18.280 --> 00:38:22.592
Sometime it's just related to their
smoking and all the other causes that we

00:38:22.592 --> 00:38:24.440
may not think as rheumatologists.

00:38:24.560 --> 00:38:28.113
So for sure,
as soon as you have a patient with ILD -

00:38:28.113 --> 00:38:32.983
proof of ILD symptomatic or not, you,
you should have at least an initial

00:38:32.983 --> 00:38:36.800
diagnosis or an initial consultation if
it's really mild.

00:38:36.960 --> 00:38:41.013
Some places or with some rheumatologists
that are comfortable to ask for the

00:38:41.013 --> 00:38:44.120
pulmonary function test and to see if
there's progression.

00:38:44.280 --> 00:38:48.144
And if you are following your patient
like this, but there's progression,

00:38:48.144 --> 00:38:51.800
you should ask back the pulmonologist to
be sure there's nothing new.

00:38:51.920 --> 00:38:54.600
And if we are at a phase,
we should treat the patient.

00:38:56.320 --> 00:38:58.240
And so it helps.

00:38:58.520 --> 00:39:01.683
It's important to get help because some
of these patient,

00:39:01.683 --> 00:39:05.720
we have to make the diagnosis,
some of them will need some investigation.

00:39:05.760 --> 00:39:09.606
As a rheumatologist,
we cannot ask and also we'll have to ask

00:39:09.606 --> 00:39:11.840
ourselves and the pulmonologist too.

00:39:12.000 --> 00:39:17.459
If the patient has bad prognosis factors
and different connective tissue disease,

00:39:17.459 --> 00:39:21.920
they have different risk factor for the
prognostic of progression.

00:39:22.120 --> 00:39:24.520
And sometimes we can know that since the
beginning.

00:39:24.520 --> 00:39:29.308
So it's important to collaborate on that
to establish which patient has a risk of

00:39:29.308 --> 00:39:32.520
further progression and how fast the
process could be.

00:39:34.280 --> 00:39:39.760
It's the same thing for both the ACR and
the ERS.

00:39:39.760 --> 00:39:44.546
So both have made a different
recommendation for bad prognostic factors

00:39:44.546 --> 00:39:49.466
to look for and then they will help us
because God knows that it's really

00:39:49.466 --> 00:39:54.320
difficult as a rheumatologist to have a
regular pulmonary function test.

00:39:54.400 --> 00:39:59.128
So with the pulmonologist that asks for
pulmonary function tests to be done

00:39:59.128 --> 00:40:01.680
regularly at like I would say really fast

00:40:02.280 --> 00:40:06.739
Like from one to the other,
it's way more easy when you have a

00:40:06.739 --> 00:40:12.332
pulmonologist in the the case and also to
ask for new chest CT scan to see the

00:40:12.332 --> 00:40:13.039
evolution.

00:40:13.040 --> 00:40:16.965
Because sometimes we are kind of full,
the pulmonary function tests are really

00:40:16.965 --> 00:40:17.760
difficult to do.

00:40:17.760 --> 00:40:21.228
But for our patients,
sometimes it looks like they they lost a

00:40:21.228 --> 00:40:22.880
lot of function in between two

00:40:22.880 --> 00:40:24.360
It's just because of the technique.

00:40:24.520 --> 00:40:30.440
So sometimes we need chest CT scan to see
if there is significant evolution.

00:40:32.200 --> 00:40:37.315
And also we have to collaborate for the
treatment because as Dr. Kolb said,

00:40:37.315 --> 00:40:41.960
sometimes we have to rely on is it more
inflammatory, more fibrotic.

00:40:42.080 --> 00:40:44.680
Sometimes we have to see the whole
picture.

00:40:44.680 --> 00:40:47.838
So the pulmonologist see the patient for
the lung part,

00:40:47.838 --> 00:40:51.560
but we are the rheumatologist to see all
the other manifestation.

00:40:51.560 --> 00:40:56.844
And sometimes we know that the connective
tissue disease or SARD that out of the

00:40:56.844 --> 00:40:58.280
lung is really active.

00:40:58.480 --> 00:41:00.120
It's going to be bad for the lung.

00:41:00.120 --> 00:41:04.404
So we have some kind of,
we have our work to say when we say well

00:41:04.404 --> 00:41:09.013
the disease is not well controlled,
so we have to change the treatment

00:41:09.013 --> 00:41:12.000
something that will not be toxic to the
lung.

00:41:12.000 --> 00:41:15.525
But still,
and this is the example from the ERS

00:41:15.525 --> 00:41:21.475
guidelines where the treatment of RA-ILD,
there's a part differentiating between

00:41:21.475 --> 00:41:24.560
patient is it - is the RA well controlled?

00:41:24.680 --> 00:41:28.815
So if the arthritis is not controlled,
for sure there's inflammation and it's

00:41:28.815 --> 00:41:31.360
going to have an impact on the ILD
progression.

00:41:31.440 --> 00:41:35.367
So even at this stage sometime it's to
start treating the RA really more

00:41:35.367 --> 00:41:38.218
aggressively,
change the treatment and it's the same

00:41:38.218 --> 00:41:40.640
for all the other connective tissue
disease.

00:41:40.640 --> 00:41:45.855
So here is the perfect example of
collaboration where you will have to say,

00:41:45.855 --> 00:41:48.120
well, maybe it's really fibrotic.

00:41:48.120 --> 00:41:52.280
But from my side when we exclude the
lungs, it's not well controlled.

00:41:52.280 --> 00:41:56.200
So you could have to add or change the
immunosuppression.

00:41:56.520 --> 00:41:59.160
So it's where we collaborate in treatment.

00:42:00.880 --> 00:42:06.894
So when you refer patient to pulmonology,
it's really important to include all the

00:42:06.894 --> 00:42:09.720
relevant information, not just ILD, RA.

00:42:09.840 --> 00:42:12.569
You know,
you have to to make it clear what is the

00:42:12.569 --> 00:42:13.640
name of the disease?

00:42:13.640 --> 00:42:15.160
What are the manifestation?

00:42:15.400 --> 00:42:17.520
We do have disease that are complicated.

00:42:17.520 --> 00:42:21.440
Lupus is is complicated,
Scleroderma is complicated.

00:42:21.440 --> 00:42:27.096
So sometimes the different manifestation
that are there are important to manifest

00:42:27.096 --> 00:42:32.614
and to say is there overlap is there -
what are the antibodies because they can

00:42:32.614 --> 00:42:37.650
have an impact on the type of ILD,
on the progression on the - there are

00:42:37.650 --> 00:42:41.719
risk factors also to mention what is the
actual treatment?

00:42:42.120 --> 00:42:44.000
What were the prior treatment?

00:42:44.000 --> 00:42:49.018
Because sometime you gave therapies that
were not tolerated or had bad side

00:42:49.018 --> 00:42:51.000
effects or were not effective.

00:42:51.200 --> 00:42:54.278
And when you go back sometime you have
like they ask you,

00:42:54.278 --> 00:42:56.932
do you want to start tocilizumab and
you're like,

00:42:56.932 --> 00:42:59.480
it really never helped the patient in the
past.

00:42:59.480 --> 00:43:00.160
We tried it.

00:43:00.160 --> 00:43:04.160
So we will not like start something that
has already been started.

00:43:04.160 --> 00:43:08.672
But sometimes you have long story with
our patient and to mention all the, the,

00:43:08.672 --> 00:43:10.760
the the prior treatment is important.

00:43:11.960 --> 00:43:15.240
Also if the disease is well controlled
and if possible,

00:43:15.240 --> 00:43:19.575
depending where you are in Canada to
include all the previous imaging and

00:43:19.575 --> 00:43:21.039
pulmonary function tests.

00:43:21.120 --> 00:43:25.815
Also cardiac ultrasound can be helpful to
the pulmonologist because it can

00:43:25.815 --> 00:43:28.320
contribute to the out of breath patient.

00:43:28.720 --> 00:43:34.520
Add the pulmonary hypertension on the ILD
and some of our disease have that.

00:43:34.520 --> 00:43:36.960
So it's important to include everything
we have.

00:43:37.080 --> 00:43:42.579
So the patient when he sees first he can
have the whole evaluation and not wait

00:43:42.579 --> 00:43:46.360
for all this information to come to the
pulmonologist.

00:43:48.720 --> 00:43:53.474
So I'll give back the control to to
Martin so we can continue with some short

00:43:53.474 --> 00:43:53.840
cases.

00:43:58.760 --> 00:44:02.000
All right, so I think, Sandra,
that was that was lovely.

00:44:03.440 --> 00:44:07.823
So, you know, I just give show you a few,
a few cases now just to put what,

00:44:07.823 --> 00:44:10.880
what Sandra and I said into a little bit
of context.

00:44:12.040 --> 00:44:14.810
If you're a rheumatologist,
you may have seen this fellow because

00:44:14.810 --> 00:44:16.280
he's a rheumatologist, Dr. Khalidi.

00:44:16.280 --> 00:44:16.840
Dr. Khalidi.

00:44:17.520 --> 00:44:19.360
And we are doing clinics together.

00:44:20.240 --> 00:44:22.714
In fact,
tomorrow morning we have a clinic where

00:44:22.714 --> 00:44:24.280
we'll see 25 patients together.

00:44:24.280 --> 00:44:28.389
And we, we,
we're doing this always in the same room

00:44:28.389 --> 00:44:33.120
at the same time because we have a very
close collaboration.

00:44:33.760 --> 00:44:37.194
So,
so this is a married couple that we saw a

00:44:37.194 --> 00:44:42.720
couple of years ago and both basically
came with the same question to me.

00:44:43.360 --> 00:44:44.560
Do they have IPF?

00:44:45.440 --> 00:44:51.960
And the the gentleman here had a very
typical clinical presentation for this.

00:44:51.960 --> 00:44:55.040
He had shortness of breath, exertion,
a dry cough.

00:44:55.040 --> 00:44:59.886
He had had some pain in his joints,
on fingers and wrists,

00:44:59.886 --> 00:45:04.240
questionable Raynaud's,
he had crackles on the exam.

00:45:05.000 --> 00:45:07.400
His skin was dried,
discoloured and cracked.

00:45:07.400 --> 00:45:10.680
But he was a mechanic,
he had worked in the steel factory.

00:45:10.680 --> 00:45:18.360
He was an ex smoker and his autoimmune
baseline work up was normal.

00:45:18.360 --> 00:45:23.124
Now, this is the CT scan,
and I don't expect you to really be able

00:45:23.124 --> 00:45:27.675
to read these images,
but this is what we call a probable usual

00:45:27.675 --> 00:45:29.879
interstitial pneumonia pattern.

00:45:29.880 --> 00:45:32.581
You see,
there's thick white lines that are in the

00:45:32.581 --> 00:45:33.800
periphery on the lungs.

00:45:33.920 --> 00:45:35.600
There's no subpleural sparing.

00:45:35.880 --> 00:45:39.592
What I showed you before for this
Scleroderma patient,

00:45:39.592 --> 00:45:44.520
you have a few cystic changes here that
are not typical honeycomb cysts.

00:45:44.680 --> 00:45:49.034
He was a smoker,
so it's probably a mix of bulla and a

00:45:49.034 --> 00:45:52.756
cyst,
but this is something that we clearly in

00:45:52.756 --> 00:45:59.407
in the whole set of his presentation said
he has IPF - this is idiopathic pulmonary

00:45:59.407 --> 00:46:00.120
fibrosis.

00:46:01.120 --> 00:46:05.480
Now just look at his wife,
she had dyspnea on exertion.

00:46:05.480 --> 00:46:07.720
So the same thing, dry cough, same thing.

00:46:07.920 --> 00:46:10.440
She had no pain in her joints or muscle.

00:46:10.720 --> 00:46:13.400
She had crackles on exam same as her
husband.

00:46:13.720 --> 00:46:18.687
She talked about a dry mouth and no dry
eyes and she was a never smoker,

00:46:18.687 --> 00:46:19.640
in the office.

00:46:20.240 --> 00:46:25.362
And here she has some abnormalities in
her baseline autoimmune panel that

00:46:25.362 --> 00:46:28.200
clearly would raise a flag from your end.

00:46:28.320 --> 00:46:32.960
A respirologist should also say, well,
there's something different.

00:46:33.000 --> 00:46:35.760
I mean, this is a woman,
she has not smoked.

00:46:36.880 --> 00:46:40.000
She has some autoimmune abnormalities.

00:46:40.000 --> 00:46:43.560
And now look at her CT scan, very similar.

00:46:43.920 --> 00:46:47.360
So it's a very similar appearance,
a little bit different to her husband.

00:46:48.080 --> 00:46:51.320
You see some areas that are the thick
white lines in the periphery.

00:46:51.760 --> 00:46:55.160
You actually see almost some honeycombing
on the one side.

00:46:55.840 --> 00:47:01.200
But it was also a little bit the central
areas of the lungs, but quite similar.

00:47:02.320 --> 00:47:06.809
She was diagnosed with Sjogren's syndrome
after this honeymoon panel with a with a

00:47:06.809 --> 00:47:08.000
salivary gland biopsy.

00:47:08.480 --> 00:47:11.160
And really this is where things are
important.

00:47:12.320 --> 00:47:14.938
They have similar symptoms,
the image looks similar,

00:47:14.938 --> 00:47:16.520
the lung function looks similar.

00:47:16.880 --> 00:47:18.200
They have two different diseases.

00:47:18.800 --> 00:47:25.085
And for a man, as I said before with IPF,
anti-inflammatory drugs are not good,

00:47:25.085 --> 00:47:27.600
they are sometimes even harmful.

00:47:28.000 --> 00:47:30.120
Antifibrotic drugs, that's what we do.

00:47:30.760 --> 00:47:35.400
In contrast, his wife, different disease,
similar appearance.

00:47:35.960 --> 00:47:39.469
We would typically choose
immunosuppressive drugs at first because

00:47:39.469 --> 00:47:40.360
she has SARD-ILD.

00:47:41.960 --> 00:47:46.601
Martin,
can I just ask for one second on the case?

00:47:46.601 --> 00:47:48.240
With your antacids

00:47:48.240 --> 00:47:54.240
is it only if there's GERD or traction
bronchiectasis that's near the esophagus

00:47:54.240 --> 00:47:55.440
or any symptoms?

00:47:55.440 --> 00:47:57.920
Or just maybe everybody should get a PPI?

00:47:57.920 --> 00:48:00.600
So they don't aspirate in their lungs
when they have ILD.

00:48:02.200 --> 00:48:02.960
Good question.

00:48:02.960 --> 00:48:07.570
So,
so the latest guidelines do not recommend

00:48:07.570 --> 00:48:11.480
routine anti-reflux medication anymore.

00:48:11.920 --> 00:48:16.537
The previous guidelines did,
and there's a little bit that came of

00:48:16.537 --> 00:48:21.568
believers or non believers in reflux
being a cofactor for lung fibrosis,

00:48:21.568 --> 00:48:23.360
particularly exacerbation.

00:48:24.040 --> 00:48:29.826
There were some studies showing that
reflux medication has no significant

00:48:29.826 --> 00:48:35.770
impact on lung function loss over time,
but it's still likely that it could

00:48:35.770 --> 00:48:37.960
contribute to exacerbations.

00:48:38.720 --> 00:48:38.840
Yeah.

00:48:38.840 --> 00:48:43.877
So what I do in my practice,
if someone has a distended esophagus or

00:48:43.877 --> 00:48:48.477
reflux symptoms all the time,
if someone has cough that is not

00:48:48.477 --> 00:48:50.959
otherwise explained, yes, I do it.

00:48:51.400 --> 00:48:56.320
But as a standard to treat the lung
function decline, I would not do it.

00:48:56.720 --> 00:48:59.280
Yeah,
but some people would still go that route.

00:48:59.280 --> 00:49:03.375
And it's not not right and not wrong
because it hasn't been really disproven

00:49:03.375 --> 00:49:04.120
either, right.

00:49:04.120 --> 00:49:09.000
And I would say for us in scleroderma,
we assume everyone has worse reflux in

00:49:09.000 --> 00:49:10.440
the general population.

00:49:10.440 --> 00:49:15.958
And so I think for the rheumatologist,
we love to use high dose PPIs if you have

00:49:15.958 --> 00:49:18.480
any symptoms of GERD and scleroderma.

00:49:19.840 --> 00:49:21.440
Yeah, I I think I would agree.

00:49:21.480 --> 00:49:21.600
Yeah.

00:49:21.600 --> 00:49:25.457
So scleroderma,
that's basically the group that always is

00:49:25.457 --> 00:49:27.320
on a PPI from my experience.

00:49:28.600 --> 00:49:31.334
OK,
now this is another case and that's I

00:49:31.334 --> 00:49:34.200
think that raises a few interesting
points.

00:49:34.200 --> 00:49:37.680
So that's a lady with rheumatoid
arthritis.

00:49:38.480 --> 00:49:42.120
I saw her first in 2018,
so five years after the diagnosis.

00:49:42.640 --> 00:49:47.817
She was stable with her rheumatoid on
methotrexate at first and then she was

00:49:47.817 --> 00:49:48.960
put on etanercept

00:49:49.520 --> 00:49:51.040
No symptoms, respiratory.

00:49:51.720 --> 00:49:53.200
She's a remote ex-smoker.

00:49:54.120 --> 00:49:58.640
Her mom had rheumatoid arthritis and
pulmonary fibrosis.

00:50:00.440 --> 00:50:05.320
So here you see some predisposition to to
interstitial lung disease.

00:50:05.640 --> 00:50:10.706
Her pulmonary function test was really
unremarkable except that mild impairment

00:50:10.706 --> 00:50:11.720
of gas transfer.

00:50:12.200 --> 00:50:13.200
That's why we saw her.

00:50:13.200 --> 00:50:18.011
So we said just stop methotrexate just in
case that was back then was still thought

00:50:18.011 --> 00:50:20.817
that methotrexate is a significant cause
of ILD,

00:50:20.817 --> 00:50:25.400
which is probably not true - probably
more preventative than causative for ILD.

00:50:26.480 --> 00:50:30.062
And you probably have to look a little
bit closer here to find some

00:50:30.062 --> 00:50:30.800
abnormalities.

00:50:30.800 --> 00:50:36.155
So that's her CT scan and you see a few
little spots of ground glass opacity here

00:50:36.155 --> 00:50:39.160
in that zone and here's you see a little
bit.

00:50:39.680 --> 00:50:39.840
Yeah.

00:50:39.840 --> 00:50:43.283
So it's really not extensive,
but she also didn't have extensive

00:50:43.283 --> 00:50:43.760
symptoms.

00:50:43.760 --> 00:50:45.680
So we don't really do much.

00:50:45.680 --> 00:50:49.480
You say well,
just in case methotrexate is a problem.

00:50:49.480 --> 00:50:55.641
So use something else if you can and keep
observing her.

00:50:55.641 --> 00:50:58.560
Now a couple of years later

00:50:59.080 --> 00:51:04.882
This lady had a mild COVID infection in
December 2022 and then her respiratory

00:51:04.882 --> 00:51:09.289
symptoms started in March the following
year, mostly cough,

00:51:09.289 --> 00:51:11.640
shortness of breath on exertion.

00:51:12.280 --> 00:51:17.818
And she was treated with antibiotics,
with prednisone and she had a flare of

00:51:17.818 --> 00:51:20.480
her RA while being on the etanercept.

00:51:20.840 --> 00:51:24.920
And her lung function is now still not
much different.

00:51:24.920 --> 00:51:30.254
But look at her CT scan and you don't
have to be a skilled radiologist to say,

00:51:30.254 --> 00:51:32.280
well, something is wrong here.

00:51:33.160 --> 00:51:37.991
You'll see now really very patchy areas
of consolidation with ground class

00:51:37.991 --> 00:51:42.371
opacity, no clear distribution,
whether it's peribronchovascular or

00:51:42.371 --> 00:51:43.079
subpleural.

00:51:43.080 --> 00:51:46.880
But that is of course something very
different.

00:51:46.880 --> 00:51:53.963
And this woman, in fact,
had a problem likely secondary to her

00:51:53.963 --> 00:51:55.200
etanercept.

00:51:56.520 --> 00:51:59.520
I know rheumatologists love anything that
blocks TNF

00:51:59.520 --> 00:52:03.186
alpha. Respirologists,
we don't like it at all because that is

00:52:03.186 --> 00:52:07.726
the drug group of the biologics and
biosimilars that has the highest rates of

00:52:07.726 --> 00:52:09.239
interstitial lung disease.

00:52:09.280 --> 00:52:13.070
Yeah, but it's of course,
a wonderful drug for the other other

00:52:13.070 --> 00:52:16.800
manifestations of rheumatoid arthritis
and other other sorts.

00:52:17.600 --> 00:52:20.314
Yeah,
but it's essentially - so remember she

00:52:20.314 --> 00:52:21.280
was predisposed.

00:52:21.280 --> 00:52:24.240
Her mom had RA,
her mom had pulmonary fibrosis.

00:52:24.760 --> 00:52:29.535
She had probably that slowly cooking
interstitial lung disease,

00:52:29.535 --> 00:52:34.610
possibly through the etanercept and then
she gets a second trigger,

00:52:34.610 --> 00:52:38.640
which is COVID and then the disease
flares up, right.

00:52:38.640 --> 00:52:44.403
And everything gone - went back to normal
with that woman by stopping etanercept to

00:52:44.403 --> 00:52:48.040
giving her a short course of prednisone
for a while.

00:52:48.040 --> 00:52:50.480
And she was started on rituximab.

00:52:50.480 --> 00:52:53.920
So as a as a respirogist,
that's the drug we really like.

00:52:54.080 --> 00:52:54.240
Yeah.

00:52:54.240 --> 00:52:58.126
I mean,
when we when we think about someone needs

00:52:58.126 --> 00:53:04.421
to be treated with the lungs and the the
rheumatoid arthritis are outside of the

00:53:04.421 --> 00:53:06.519
lungs, we love three drugs:

00:53:06.520 --> 00:53:09.620
The one is rituximab,
the other is tocilizumab,

00:53:09.620 --> 00:53:12.720
and the third is a new group of JAK
inhibitors.

00:53:12.720 --> 00:53:12.920
Yeah.

00:53:12.920 --> 00:53:16.040
And those are the drugs that are probably
good for both fronts.

00:53:16.600 --> 00:53:21.887
Respirologists also likes mycophenolate
but that doesn't do too much to the SARD

00:53:21.887 --> 00:53:24.760
unless you have a vasculitis or a
myositis.

00:53:25.000 --> 00:53:27.520
Yeah, but that, that's usually what we do.

00:53:28.200 --> 00:53:31.700
So that's, you know,
just I I give it back to Janet now,

00:53:31.700 --> 00:53:32.560
and I shut up.

00:53:32.880 --> 00:53:33.160
Yeah.

00:53:33.320 --> 00:53:34.920
No, no, not to shut up at all.

00:53:34.920 --> 00:53:40.860
I was just going to say that I always
used to in my talk say TNFs were lung

00:53:40.860 --> 00:53:41.720
unfriendly.

00:53:42.040 --> 00:53:44.640
And I mean, either way, she broke through.

00:53:44.640 --> 00:53:47.000
I mean, as you say,
COVID was the exacerbator.

00:53:47.000 --> 00:53:51.787
So you'd change because she not just
flared, her lungs got way worse,

00:53:51.787 --> 00:53:53.360
but her joints as well.

00:53:53.360 --> 00:53:54.280
So you'd move on.

00:53:54.280 --> 00:54:01.108
But just for the the those listening,
there was a large study out of the US VA

00:54:01.108 --> 00:54:01.800
network.

00:54:01.800 --> 00:54:06.240
So the the Vets - Veteran Affairs
hospital is outpatient.

00:54:06.240 --> 00:54:08.925
So they're primarily men and they mostly
smoke,

00:54:08.925 --> 00:54:13.624
so they're not like our usual RA patients
because they are more heavily weighted to

00:54:13.624 --> 00:54:14.520
men and smoking.

00:54:15.040 --> 00:54:19.730
But what they found is if you adjusted
for a whole bunch of factors,

00:54:19.730 --> 00:54:24.897
because TNFs are often our first line
advanced therapy when someone flares,

00:54:24.897 --> 00:54:29.520
at least for many years has been the case
when they flare their RA.

00:54:29.760 --> 00:54:34.340
So the patients years ago that got on a
TNF were the worst because they,

00:54:34.340 --> 00:54:36.160
we didn't have other options.

00:54:36.360 --> 00:54:38.080
So they adjusted for a bunch of stuff.

00:54:38.080 --> 00:54:40.440
And so it looked like TNFs were worse.

00:54:40.440 --> 00:54:43.291
And when they adjusted for all sorts of
confounding,

00:54:43.291 --> 00:54:46.680
they look like they were as bad or as
good as everything else.

00:54:46.920 --> 00:54:49.880
But I think either way,
the point is you're going to move on

00:54:49.880 --> 00:54:51.240
because the person worsened.

00:54:51.520 --> 00:54:56.520
So I think that's that's a great case for
us for learning and looking.

00:54:56.520 --> 00:54:58.480
And the second hit hypothesis