Understanding and Applying the 2025 “ILAs” (Interstitial lung abnormalities) Guidelines in Clinical Practice
00:00:02.370 --> 00:00:05.687
Alright, I think we'll,
we'll slowly get underway here.
00:00:05.687 --> 00:00:09.181
I'm sure more people are going to be
joining as we go. So,
00:00:09.181 --> 00:00:12.854
I'll start off just by introducing myself.
I'm Chris Ryerson.
00:00:12.854 --> 00:00:17.770
I'm in the Department of Medicine at UBC
and do a lot of interstitial lung disease
00:00:17.770 --> 00:00:22.331
work and had the pleasure of being
involved in the ILA clinical statement or
00:00:22.331 --> 00:00:26.477
the, the guidelines for this.
So little bit of insider knowledge into
00:00:26.477 --> 00:00:31.156
what went into this document and very
pleased to introduce somebody else who's
00:00:31.156 --> 00:00:34.000
going to talk to us about this,
which is great.
00:00:34.780 --> 00:00:38.262
And pleased to introduce Doctor Amanda
Grant-Orser.
00:00:38.262 --> 00:00:43.486
I'll call her Amanda throughout. So,
Amanda is a clinical assistant professor
00:00:43.486 --> 00:00:48.040
at the University of Calgary.
She's a director of the ILD clinic at
00:00:48.040 --> 00:00:53.532
Foothills Medical Centre and quickly has
gained a national and even international
00:00:53.532 --> 00:00:58.153
reputation in the field of ILD.
She did her fellowship in ILD at the
00:00:58.153 --> 00:01:02.573
University of Calgary after doing some of
her training in London,
00:01:02.573 --> 00:01:06.190
ON as well as in Ireland.
Her research focuses on ILA
00:01:06.270 --> 00:01:11.573
as well as familial ILD and prognostic
biomarkers and she's dedicated her career
00:01:11.573 --> 00:01:16.549
to improving the outcomes and quality of
life for patients living with ILD.
00:01:16.549 --> 00:01:20.608
So very pleased to have Amanda join us to
speak today. First,
00:01:20.608 --> 00:01:25.388
a couple of housekeeping things.
Your cameras are going to be turned off
00:01:25.388 --> 00:01:29.251
throughout the talk.
So you won't actually be able to turn
00:01:29.251 --> 00:01:32.852
your camera on.
Your microphones are going to be muted
00:01:32.852 --> 00:01:36.650
throughout the talk,
but there is going to be an open mic
00:01:36.650 --> 00:01:39.400
Q&A session at the end of the session.
00:01:39.620 --> 00:01:43.014
In the meantime,
you can also put your questions into the
00:01:43.014 --> 00:01:47.695
chat box and I will be collating those
and posing some of those towards the end
00:01:47.695 --> 00:01:51.792
as well, if people don't want to speak up.
At the end of the session,
00:01:51.792 --> 00:01:56.532
there will be an evaluation form that's
shown on the screen and please fill that
00:01:56.532 --> 00:02:00.394
out if you have a moment to.
We do have a steering committee that
00:02:00.394 --> 00:02:05.017
decides what topics to present and what
speaker to invite and we take feedback
00:02:05.017 --> 00:02:07.300
from these evaluations quite seriously.
00:02:08.520 --> 00:02:12.597
Alright, next slide.
We have received support for this from
00:02:12.597 --> 00:02:15.928
Boehringer.
They provide the logistical support,
00:02:15.928 --> 00:02:21.297
but they didn't provide any input on the
content that will be discussed today.
00:02:21.297 --> 00:02:25.171
We've received some speaking honoraria
well, Amanda has,
00:02:25.171 --> 00:02:27.890
about this talk from Boehringer as well.
00:02:29.380 --> 00:02:30.130
Next slide,
00:02:31.780 --> 00:02:37.590
this is Doctor Grant-Orser's conflicts of
interest and next slide is is mine.
00:02:38.460 --> 00:02:41.438
And with that,
I'll turn the the microphone over to
00:02:41.438 --> 00:02:41.840
Amanda.
00:02:43.440 --> 00:02:48.269
Alright, perfect. Thanks, Doctor Ryerson,
and thanks so much everyone for attending
00:02:48.269 --> 00:02:52.121
on a Wednesday afternoon evening,
wherever you are in the country.
00:02:52.121 --> 00:02:55.571
I'm really excited to talk about these
2025 ILA guidelines.
00:02:55.571 --> 00:02:59.941
And over the next about 40 minutes,
hopefully saving about 15 for questions
00:02:59.941 --> 00:03:03.045
and answers.
We'll talk about some key updates of the
00:03:03.045 --> 00:03:07.703
guidelines and start off with a little
bit about how we got to the guidelines in
00:03:07.703 --> 00:03:10.922
the 1st place.
I'll talk about a few clinical scenarios
00:03:10.922 --> 00:03:14.890
where I think early recognition of ILAs
may impact patient outcomes,
00:03:15.430 --> 00:03:18.061
You know,
how we can guide timely referral to the
00:03:18.061 --> 00:03:21.534
right place, right time.
And then we'll also talk about some case
00:03:21.534 --> 00:03:23.640
discussions to guide patient management.
00:03:24.960 --> 00:03:28.249
So to start off with,
we'll start off with the a polling
00:03:28.249 --> 00:03:32.463
question. Based on your current knowledge,
which case represents an ILA?
00:03:32.463 --> 00:03:36.734
So the first one is a 54-year-old male
with renal colic presenting to the
00:03:36.734 --> 00:03:40.370
emergency department,
has a CT abdo pelvis that gets the lower
00:03:40.370 --> 00:03:43.545
images of the thorax.
He does have a family history of
00:03:43.545 --> 00:03:47.758
interstitial lung disease and might have
some mild respiratory symptoms.
00:03:47.758 --> 00:03:51.105
The second case,
B is an 83-year-old gentleman undergoing
00:03:51.105 --> 00:03:55.146
nodule follow-up CT scans.
He does have a bit of dyspnea at baseline,
00:03:55.146 --> 00:03:56.820
but normal pulmonary function
00:03:56.900 --> 00:04:01.366
testing and the third - 68 year old
female with a CT abdomen to investigate a
00:04:01.366 --> 00:04:05.031
renal mass, same thing.
There's some lower images of her lungs.
00:04:05.031 --> 00:04:08.582
She's asymptomatic and has normal
pulmonary function testing.
00:04:08.582 --> 00:04:11.560
So one of these, all of the above,
or none of them.
00:04:13.410 --> 00:04:15.660
So I'll put up this. There we go.
00:04:17.380 --> 00:04:19.320
Hopefully people on their phones can
00:04:20.360 --> 00:04:21.390
click on this.
00:04:29.690 --> 00:04:30.730
All right, Super.
00:04:37.300 --> 00:04:39.300
I'll give it another second here.
00:04:40.550 --> 00:04:45.670
Hopefully everybody can see the end of
the poll if I do that, share results.
00:04:50.510 --> 00:04:54.190
OK,
So hopefully people can see that a little
00:04:54.190 --> 00:04:58.510
bit of a nice split.
So 25% thought all of the above,
00:04:58.510 --> 00:05:02.030
50% thought case C and a few for case B
and
00:05:02.920 --> 00:05:03.350
A.
00:05:04.400 --> 00:05:05.100
All right.
00:05:07.640 --> 00:05:10.962
So before we really get into the details
of the, the guidelines,
00:05:10.962 --> 00:05:15.001
I think it's important to tell a little
bit of a story about the background of
00:05:15.001 --> 00:05:17.454
this term,
interstitial lung abnormalities and,
00:05:17.454 --> 00:05:21.288
and kind of when it first came on the
scene and some data behind why these
00:05:21.288 --> 00:05:23.180
guidelines were felt to be important.
00:05:24.280 --> 00:05:29.210
So interstitial lung abnormalities was
first really put forward in 2020 as
00:05:29.210 --> 00:05:34.207
incidental CT radiologic parenchymal
abnormalities detected in patients not
00:05:34.207 --> 00:05:38.678
known to have lung disease.
And I think the goal of this originally
00:05:38.678 --> 00:05:43.938
Fleischner paper was to try and somewhat
harmonize some of the other terms that
00:05:43.938 --> 00:05:48.474
have been floating around,
things like incidental ILD, pre-clinical,
00:05:48.474 --> 00:05:51.827
my favorite,
geriatric ID to try and harmonize the
00:05:51.827 --> 00:05:56.101
term in part to guide clinicians,
also to guide radiologists and
00:05:56.101 --> 00:05:56.890
researchers.
00:05:57.760 --> 00:06:03.106
So many of you might be familiar with
this 2020 Fleischner Society position
00:06:03.106 --> 00:06:08.241
paper that gave a really nice definition
of ILA's as being non-dependent
00:06:08.241 --> 00:06:12.111
abnormalities affecting more than 5% of
any lung zone.
00:06:12.111 --> 00:06:17.879
And the abnormalities were ground glass,
reticulation, lung distortion, traction,
00:06:17.879 --> 00:06:21.960
bronchiectasis,
honeycombing and non-emphysematous cysts.
00:06:22.610 --> 00:06:26.893
And the original ILA recommendations was
that these are supposed to be in
00:06:26.893 --> 00:06:31.118
individuals in whom interstitial lung
disease is not suspected and often
00:06:31.118 --> 00:06:32.160
incidental finding
00:06:33.300 --> 00:06:37.373
this position paper went on to define it
into 3 sub categories.
00:06:37.373 --> 00:06:42.210
They're pretty self-explanatory.
Non-subpleural, usually some ground glass,
00:06:42.940 --> 00:06:45.665
subpleural,
non-fibrotic and then subpleural,
00:06:45.665 --> 00:06:48.449
fibrotic.
And part of this had to do with some
00:06:48.449 --> 00:06:52.300
prognostic indicators for each of these
different subcategories.
00:06:53.540 --> 00:06:56.715
So how does this kind of fit into the
realm of ILD?
00:06:56.715 --> 00:07:00.746
I think many of us are familiar with this
diagram here, you know,
00:07:00.746 --> 00:07:05.449
the different trajectories that patients
with pulmonary fibrosis often take.
00:07:05.449 --> 00:07:08.502
Some patients decline slowly,
some patients fast,
00:07:08.502 --> 00:07:13.083
some in a stepwise manner. And so,
we're often meeting patients, you know,
00:07:13.083 --> 00:07:17.969
at the time of symptom onset or diagnosis
after usually a significant amount of
00:07:17.969 --> 00:07:20.290
fibrosis has already been established.
00:07:21.570 --> 00:07:25.180
And so the thought is,
the disease process starts years,
00:07:25.180 --> 00:07:28.601
months beforehand. And so again,
just honing in here,
00:07:28.601 --> 00:07:33.415
this is when we meet patients and it's
thought that these interstitial lung
00:07:33.415 --> 00:07:38.420
abnormalities probably start years ahead
of time and not everybody with ILA is
00:07:38.420 --> 00:07:42.537
necessarily going to go on to develop
interstitial lung disease.
00:07:42.537 --> 00:07:46.465
Some of these ILAs resolve,
some patients have a mild course,
00:07:46.465 --> 00:07:49.569
some unfortunately go on to develop the
disease.
00:07:49.569 --> 00:07:53.750
We're most familiar with idiopathic
pulmonary fibrosis, which has
00:07:53.840 --> 00:07:57.539
quite a bit of mortality and morbidity
associated with it.
00:07:57.539 --> 00:08:01.301
And I think really what the goal of this,
you know, ILA is,
00:08:01.301 --> 00:08:06.380
is an opportunity for early detection to
try and risk stratify these patients to
00:08:06.380 --> 00:08:10.205
determine who will progress and try and
answer the question,
00:08:10.205 --> 00:08:14.030
if we intervene early,
can we prevent irreversible fibrosis?
00:08:15.390 --> 00:08:18.917
And So what about ILAs just by themselves
in terms of, you know,
00:08:18.917 --> 00:08:22.390
do they matter before it's actually
determined to be a disease?
00:08:23.190 --> 00:08:25.838
Let's start off with talking about
prevalence.
00:08:25.838 --> 00:08:30.233
So these are actually really common.
So this is some work that our group did,
00:08:30.233 --> 00:08:34.346
which is a systematic review and meta
analysis of all of the lung cancer
00:08:34.346 --> 00:08:38.009
screening programs that have been
published up until about 2023.
00:08:38.009 --> 00:08:41.897
And what we found was that ILAs were
prevalent in about 7% of cases,
00:08:41.897 --> 00:08:42.629
so quite high
00:08:44.100 --> 00:08:46.361
In Canada,
I think this is relevant because there
00:08:46.361 --> 00:08:50.161
are some provinces that are already doing
lung cancer screening and more kind of on
00:08:50.161 --> 00:08:53.509
the way. And so I think, you know,
even if we're not sure what to do with
00:08:53.509 --> 00:08:56.268
ILAs and we'll get to that in the
management side of things,
00:08:56.268 --> 00:08:58.711
I think these referrals are coming to
respirologists.
00:08:58.711 --> 00:09:01.290
And so having some guidance is really,
really important.
00:09:02.760 --> 00:09:05.322
What about in general population
screening?
00:09:05.322 --> 00:09:10.098
So this is kind of lung health screenings
that some communities and countries do.
00:09:10.098 --> 00:09:12.720
Same thing,
prevalence was quite high at 7%.
00:09:13.520 --> 00:09:18.511
And then I think maybe unsurprisingly if
you actually screen really at risk
00:09:18.511 --> 00:09:21.663
cohorts,
so this is familial pulmonary fibrosis
00:09:21.663 --> 00:09:26.983
cohorts where you screen thought to be
unaffected first-degree family relatives,
00:09:26.983 --> 00:09:30.135
the prevalence is really high at about
26%. So,
00:09:30.135 --> 00:09:34.929
one in four at risk relatives might have
these radiologic abnormalities.
00:09:37.430 --> 00:09:40.093
And so I've probably alluded to this,
you know,
00:09:40.093 --> 00:09:44.200
the thought is that some of these
patients like we have early preclinical
00:09:44.200 --> 00:09:48.418
interstitial lung disease and what we
know is that many of those with these
00:09:48.418 --> 00:09:53.025
fibrotic changes will progress over time.
It really depends on how long you follow
00:09:53.025 --> 00:09:55.689
them for. So, you know,
varying follow up time,
00:09:55.689 --> 00:09:58.575
if you follow them for two years,
less progressive,
00:09:58.575 --> 00:10:01.627
if you follow them longer to five years
more progress.
00:10:01.627 --> 00:10:06.123
And this is just a nice example here of
some ILA progression where there's some,
00:10:06.123 --> 00:10:08.676
you know,
a little bit of ground glass in the
00:10:08.676 --> 00:10:09.120
picture.
00:10:09.190 --> 00:10:12.438
And then on follow up,
there's been some evolution to some
00:10:12.438 --> 00:10:13.760
traction bronchiectasis.
00:10:15.490 --> 00:10:18.633
This is directly from the guidelines as
supplement.
00:10:18.633 --> 00:10:23.590
So they looked at all of the studies that
looked at progression and found similar
00:10:23.590 --> 00:10:28.244
findings that about 46% of the the data
that helped inform these guidelines,
00:10:28.244 --> 00:10:30.299
there's ILA progression over time.
00:10:32.980 --> 00:10:37.083
The other really striking thing about
ILA's is that there's an increased risk
00:10:37.083 --> 00:10:39.555
of all-cause mortality and a pretty,
you know,
00:10:39.555 --> 00:10:41.975
significant that we need to pay attention
to.
00:10:41.975 --> 00:10:46.500
And this is true across different cohorts,
lung cancer screening, general population.
00:10:48.310 --> 00:10:52.504
The other important thing is that this
can affect treatment for things. So again,
00:10:52.504 --> 00:10:54.959
coming back to that lung cancer side of
things,
00:10:54.959 --> 00:10:58.642
we know that ILAs impact the risk of
things like radiation pneumonitis,
00:10:58.642 --> 00:11:02.580
immune checkpoint inhibitor pneumonitis
and an increased risk of lung cancer
00:11:02.580 --> 00:11:05.650
mortality overall. So again,
something to pay attention to.
00:11:08.190 --> 00:11:11.061
And so, you know,
the big question is who is going to
00:11:11.061 --> 00:11:14.305
progress and when?
And so there's been a quite a bit of work
00:11:14.305 --> 00:11:18.453
looking at trying to answer this question
and I'll just highlight a couple of
00:11:18.453 --> 00:11:19.570
relevant data points.
00:11:20.970 --> 00:11:24.163
So in terms of clinical predictors of
progression,
00:11:24.163 --> 00:11:29.172
so this was a fairly large study that
looked at the AGES-Reykjavik longitudinal
00:11:29.172 --> 00:11:32.553
birth cohort.
So essentially you know normal patients
00:11:32.553 --> 00:11:37.061
that it gets CT scans vote every five
years for life and they looked at
00:11:37.061 --> 00:11:42.070
patients that had violas with progression
compared to patients who had no Ilas.
00:11:42.790 --> 00:11:46.789
And so they looked at some kind of
clinical slash biomarker predictors that
00:11:46.789 --> 00:11:48.000
were interested in ILD.
00:11:48.830 --> 00:11:52.955
So I think unsurprisingly,
patients that have the same risk factors
00:11:52.955 --> 00:11:55.806
for things like idiopathic pulmonary
fibrosis,
00:11:55.806 --> 00:12:00.053
the MUC5B genotype and age had an
increased risk of both having ILAs,
00:12:00.053 --> 00:12:05.088
that's on the right. And if you had ILAs,
there's an increased risk of progression
00:12:05.088 --> 00:12:08.425
on the left.
The MUC5B genotype increases your risk of
00:12:08.425 --> 00:12:13.157
having idiopathic pulmonary fibrosis.
I think there's a lot of enthusiasm for
00:12:13.157 --> 00:12:16.615
this, you know,
let's call it a biomarker for predicting
00:12:16.615 --> 00:12:20.680
the risk of who's going to get IPF,
but it's not really clinically
00:12:20.760 --> 00:12:23.163
used at this point.
It's still very much a research tool,
00:12:23.163 --> 00:12:26.230
but age is something, you know,
as clinicians we could use in our clinic.
00:12:27.340 --> 00:12:31.612
What else could we use in our clinic?
What else do we ask patients about? Well,
00:12:31.612 --> 00:12:34.121
things like sex, body mass index,
and smoking.
00:12:34.121 --> 00:12:37.539
While some of these certainly increase
your risk of having ILA,
00:12:37.539 --> 00:12:41.971
they weren't very good at predicting who
was going to progress with ILAs once they
00:12:41.971 --> 00:12:43.360
were actually established.
00:12:45.360 --> 00:12:48.410
So [other] things can be used to project
who's going to progress?
00:12:49.670 --> 00:12:52.635
What about radiologic predictors of
progression?
00:12:52.635 --> 00:12:56.872
So this is kind of collated couple of
studies that have looked at the
00:12:56.872 --> 00:12:59.777
radiologic markers and and risk of
progression.
00:12:59.777 --> 00:13:02.440
So starting from the bottom up,
subpleural,
00:13:02.440 --> 00:13:06.798
nonfibrotic reticulation might increase
your risk of progression a bit.
00:13:06.798 --> 00:13:11.579
If you have a definite UIP pattern as per
the guidelines, definitely increase.
00:13:11.579 --> 00:13:16.361
And if you have very clear fibrosis,
traction, bronchiectasis or honeycombing,
00:13:16.361 --> 00:13:20.840
this is kind of removing some of the
radiologic distribution definitions,
00:13:20.990 --> 00:13:23.534
really high risk of progression.
And so again,
00:13:23.534 --> 00:13:27.486
this is just trying to highlight that no
matter how you define fibrosis,
00:13:27.486 --> 00:13:31.330
it's probably abnormal and it's probably
going to get worse over time.
00:13:32.840 --> 00:13:35.948
So just to hone in on those factors a
little bit, again,
00:13:35.948 --> 00:13:40.148
this is kind of separating out some of
those radiologic risks of prediction.
00:13:40.148 --> 00:13:43.311
We know that if there's a lower lobe
predominant pattern,
00:13:43.311 --> 00:13:44.730
there's an increased risk.
00:13:45.610 --> 00:13:48.370
If there's subpleural reticulation,
there's an increased risk.
00:13:49.080 --> 00:13:51.940
If there's traction bronchiectasis,
definitely an increased risk
00:13:52.600 --> 00:13:55.902
and if there's honeycombing,
you likely almost certainly will get
00:13:55.902 --> 00:13:58.154
worse. Again,
the question is over how long?
00:13:58.154 --> 00:14:02.157
So the study that I took this data from
was over 5 year follow up and everybody
00:14:02.157 --> 00:14:05.160
who had honeycombing had progression at
the five year mark.
00:14:08.080 --> 00:14:10.288
OK.
So what doesn't increase your risk of
00:14:10.288 --> 00:14:10.920
progression?
00:14:11.740 --> 00:14:15.445
So nonemphysematous cysts probably don't
increase your risk of progression.
00:14:15.445 --> 00:14:19.297
This is something that was included in
the previous Fleischner paper and we'll
00:14:19.297 --> 00:14:22.173
see that has been taken out of the most
recent guidelines.
00:14:22.173 --> 00:14:25.927
Things like centrilobular nodules don't
increase your risk of progression if
00:14:25.927 --> 00:14:29.633
you've got an indeterminate pattern for
UIP and non-subpleural ground glass
00:14:29.633 --> 00:14:32.071
opacities,
those all are pretty good markers that
00:14:32.071 --> 00:14:33.680
hopefully things won't get worse.
00:14:35.850 --> 00:14:38.857
OK, so coming back to, you know,
time to progression.
00:14:38.857 --> 00:14:43.313
So we know that there's a sanity ILA,
but I think it would be really helpful to
00:14:43.313 --> 00:14:46.376
know kind of when that progression is
going to happen.
00:14:46.376 --> 00:14:50.610
And so this is a really nice study that
that tried to answer that question.
00:14:51.350 --> 00:14:56.269
This looked at about 4700 patients in a
healthy lung health screening program,
00:14:56.269 --> 00:15:01.001
and they found that there was about a 7%
prevalence of ILAs, which is nice,
00:15:01.001 --> 00:15:05.235
it matched our previous data and looked
at those who had, you know,
00:15:05.235 --> 00:15:08.847
true ILAs not indeterminate for ILAs.
We'll come to this,
00:15:08.847 --> 00:15:12.645
but sometimes it's hard based on
inspiration of the CT scan,
00:15:12.645 --> 00:15:15.945
if it's low dose, high resolution,
things like that,
00:15:15.945 --> 00:15:20.553
whether it's a true ILA or not.
So a lot of these studies sometimes have,
00:15:20.553 --> 00:15:23.480
you know,
hedging your bets and indeterminate,
00:15:23.560 --> 00:15:25.955
for ILA.
This one really tried to include those
00:15:25.955 --> 00:15:29.000
that had clear features of an
interstitial lung abnormality.
00:15:30.360 --> 00:15:35.140
They looked at 239 of these patients and
they removed those patients that had a
00:15:35.140 --> 00:15:38.068
suspected undiagnosed interstitial lung
disease.
00:15:38.068 --> 00:15:42.789
And interstitial lung disease in this
case was defined as those with extensive
00:15:42.789 --> 00:15:45.837
CT abnormalities.
So basically bilateral fibrosis,
00:15:45.837 --> 00:15:50.139
over 5% of three or more lung zones were
classified as having suspected
00:15:50.139 --> 00:15:53.784
interstitial lung disease.
And basically they followed these
00:15:53.784 --> 00:15:56.712
patients.
Usually they'd have a CT scan at about
00:15:56.712 --> 00:16:01.493
every two years and for the purpose of
this study they wanted to have people to
00:16:01.493 --> 00:16:02.330
have a CT scan
00:16:02.400 --> 00:16:03.850
and at least the five year follow up.
00:16:06.620 --> 00:16:11.800
And what was pretty striking was over the
course of the the entire study,
00:16:11.800 --> 00:16:15.300
81% of patients had some form of ILA
progression.
00:16:16.520 --> 00:16:22.410
ILAs progressed to a clear UIP pattern in
17% of patients.
00:16:23.170 --> 00:16:25.746
And this is I think the really helpful
data point,
00:16:25.746 --> 00:16:28.526
the median time to ILA progression was 3.
2 years. So,
00:16:28.526 --> 00:16:31.052
I think this is the first time we said,
you know,
00:16:31.052 --> 00:16:33.680
this is when we start to see some of
these changes.
00:16:34.620 --> 00:16:39.126
What was helpful from a, you know,
an a understanding the Natural History of
00:16:39.126 --> 00:16:42.578
this process number,
was that the progression to UIP could
00:16:42.578 --> 00:16:45.856
take upwards of close to 12 years. So,
you know, again,
00:16:45.856 --> 00:16:48.723
I think we have all seen patients in our
clinic.
00:16:48.723 --> 00:16:53.112
You look back on a CT 5 plus years ago
and there's probably something that
00:16:53.112 --> 00:16:55.746
started.
How we translate that into clinical
00:16:55.746 --> 00:16:59.081
practice is a little bit harder to
understand. You know,
00:16:59.081 --> 00:17:03.705
following asymptomatic patients for 12
years might be difficult when we've got
00:17:03.705 --> 00:17:06.280
already pretty busy clinical practices,
but
00:17:06.350 --> 00:17:10.005
happy to have that discussion at the
Q&A section. But really, you know,
00:17:10.005 --> 00:17:13.708
I think this is important data showing
that at least on a median time frame,
00:17:13.708 --> 00:17:16.210
it takes three years for these changes to
progress.
00:17:19.180 --> 00:17:24.200
OK, well, you know, we've identified ILAs.
Let's talk about treatment, with what?
00:17:24.200 --> 00:17:27.935
and for who? So right now,
no studies to date have evaluated
00:17:27.935 --> 00:17:30.751
treatment for ILA. I think there's,
you know,
00:17:30.751 --> 00:17:35.833
a lot of enthusiasm and there's a theory
that earlier treatment of ILD should lead
00:17:35.833 --> 00:17:39.874
to better outcomes. So,
what treatments do we really focus on for
00:17:39.874 --> 00:17:43.854
at least ILD right now,
the established therapies, antifibrotic.
00:17:43.854 --> 00:17:47.038
Sorry if this doesn't project very well.
Of course,
00:17:47.038 --> 00:17:49.120
there's nintedanib and pirfenidone
00:17:49.960 --> 00:17:52.734
you know,
we've seen that they reduce FVC decline
00:17:52.734 --> 00:17:56.063
over time and you know,
we think that they have a mortality
00:17:56.063 --> 00:18:00.557
benefit of antifibrotic therapy as well.
There's lots of kind of systematic meta
00:18:00.557 --> 00:18:04.274
analyses in real world studies.
This study in particular was a bit
00:18:04.274 --> 00:18:08.546
provocative because it did talk about
whether or not there was a true signal
00:18:08.546 --> 00:18:12.874
for mortality benefit when you took out
immortal time bias. And so, you know,
00:18:12.874 --> 00:18:17.201
we certainly hope that we're doing right
by our patients and making them live
00:18:17.201 --> 00:18:17.590
longer.
00:18:18.360 --> 00:18:20.840
Of course, these are wonderful drugs.
They're what we have.
00:18:20.840 --> 00:18:24.190
They also come with some side effects and
costs that we have to be cognizant of.
00:18:25.500 --> 00:18:30.073
OK, what about preventative therapies?
So this was a nice recent publication
00:18:30.073 --> 00:18:35.002
that looked at the impact of vaccination
in fibrotic interstitial lung disease and
00:18:35.002 --> 00:18:39.337
the risk of mortality in COVID.
And the main take away point was that if
00:18:39.337 --> 00:18:42.485
you were vaccinated from COVID and
contracted COVID,
00:18:42.485 --> 00:18:46.880
you had less risk of mortality.
They also looked at immunosuppression and
00:18:46.880 --> 00:18:51.513
there wasn't really a signal of harm or
benefit if you were immunosuppressed.
00:18:51.513 --> 00:18:55.789
But again, this is, you know,
a really nice paper that shows that there
00:18:55.789 --> 00:18:56.679
is some benefit
00:18:56.750 --> 00:19:00.460
hopefully in our patients for
vaccinations and the fibrotic ILD space.
00:19:02.260 --> 00:19:05.180
And then of course,
what about emerging therapies?
00:19:05.180 --> 00:19:09.075
What's coming to market soon?
I think we're all very excited at the
00:19:09.075 --> 00:19:13.370
results of the FIBRONEER trials.
This one data point in particular is from
00:19:13.370 --> 00:19:14.230
the PPF cohort.
00:19:15.090 --> 00:19:17.894
And you know,
this is the first trial that's really
00:19:17.894 --> 00:19:21.455
shown as a secondary endpoint.
But in a randomized control trial,
00:19:21.455 --> 00:19:25.662
there was a benefit for death in our ILD
population, which is just, you know,
00:19:25.662 --> 00:19:28.737
so exciting. So,
I think there's some emerging therapies
00:19:28.737 --> 00:19:32.944
that are coming to market that, you know,
hopefully will be beneficial to our
00:19:32.944 --> 00:19:33.430
patients.
00:19:34.920 --> 00:19:38.741
And I'd like to frame it kind of like
statin therapy. You know,
00:19:38.741 --> 00:19:43.399
I'm hopeful that within my career we get
a medication that is considered both
00:19:43.399 --> 00:19:46.982
primary prevention and secondary
prevention. And of course,
00:19:46.982 --> 00:19:49.669
we have to think about all things like
cost,
00:19:49.669 --> 00:19:53.670
how much is it going to reduce the risk,
safety, things like that.
00:19:53.670 --> 00:19:58.268
But I really hope that we can find
something like that for interstitial lung
00:19:58.268 --> 00:20:00.895
disease.
And I think focusing on ILAs is an
00:20:00.895 --> 00:20:02.150
opportunity for that.
00:20:04.400 --> 00:20:10.080
So without further ado,
I'll get into our guidelines.
00:20:13.570 --> 00:20:18.007
So this was the approach to the
evaluation and management of interstitial
00:20:18.007 --> 00:20:21.725
lung abnormalities.
It was an ATS Society clinical statement.
00:20:21.725 --> 00:20:25.142
There was 4 Co-chairs,
well recognized in the field, Dr.
00:20:25.142 --> 00:20:30.060
Podolanczuk, Dr. Hunninghake, our own Dr.
Chris Ryerson, as well as Dr. Schwartz.
00:20:32.150 --> 00:20:36.103
And so this was a committee that was
combined with both clinical and
00:20:36.103 --> 00:20:39.712
methodologic experts.
There was also two patient partners that
00:20:39.712 --> 00:20:42.863
were involved in it.
The questions were drafted by the
00:20:42.863 --> 00:20:45.957
Co-chairs and then put forward to
committee approval.
00:20:45.957 --> 00:20:50.540
There was an evidence synthesis process
for each of the PICO questions that was
00:20:50.540 --> 00:20:54.951
generated and then basically it was kind
of consensus by discussion with the
00:20:54.951 --> 00:20:59.248
voting practice afterwards either to
accept or not accept a suggestion and
00:20:59.248 --> 00:21:03.545
over 70% agreement was required.
This underwent several peer review cycles
00:21:03.545 --> 00:21:03.660
by
00:21:03.810 --> 00:21:07.124
experts in the field,
and there is a planned three-year review
00:21:07.124 --> 00:21:10.070
by the ATS to see if there's an update
that's required.
00:21:13.840 --> 00:21:18.037
In terms of core concepts.
Before we get into some of the details,
00:21:18.037 --> 00:21:21.921
the authors, you know,
off the bat recognize that normal lung
00:21:21.921 --> 00:21:25.680
ILA and ILD's are likely a spectrum of
changes in the lung.
00:21:27.020 --> 00:21:30.663
One thing that was important was the
incidental requirement that was put
00:21:30.663 --> 00:21:34.157
forward in the Fleischner Society
position paper was removed from the
00:21:34.157 --> 00:21:36.702
definition.
I think - #1 radiologists don't always
00:21:36.702 --> 00:21:40.246
know why the scan is being done.
I certainly just sometimes click off,
00:21:40.246 --> 00:21:44.389
you know, shortness of breath or you know,
our colleagues might click off nodule -
00:21:44.389 --> 00:21:46.984
follow up,
but they might have shortness of breath.
00:21:46.984 --> 00:21:50.627
So all of that information might not
actually be there for radiologists.
00:21:50.627 --> 00:21:53.572
And it, you know,
the thought was that it makes a bit more
00:21:53.572 --> 00:21:55.070
clinical sense to remove that.
00:21:56.290 --> 00:22:00.658
The authors also recognize that some of
these recommendations are trying to
00:22:00.658 --> 00:22:04.395
balance the sensitivity and specificity
for disease progression.
00:22:04.395 --> 00:22:09.051
I think that's what we're all worried
about is the the progressing type of ILAs.
00:22:09.051 --> 00:22:13.478
They also acknowledge that not all
disease necessarily needs treatment if it
00:22:13.478 --> 00:22:16.870
meets that ILD definition that the
guideline puts forward.
00:22:18.230 --> 00:22:22.662
They acknowledge that all imaging is not
equal and if there's a question if this
00:22:22.662 --> 00:22:26.712
is an ILA or indeterminate for ILA,
probably getting a high resolution CT
00:22:26.712 --> 00:22:28.190
with prone images is ideal.
00:22:28.960 --> 00:22:32.598
And then they also comment that some of
the recommendations are there to really
00:22:32.598 --> 00:22:35.600
stimulate some evidence generation in a
field that is pretty new.
00:22:36.490 --> 00:22:39.253
Finally,
and I think really importantly was there
00:22:39.253 --> 00:22:43.011
was a patient voice component and the
patients really thought early
00:22:43.011 --> 00:22:46.990
identification was important and so that
was prioritized in this paper.
00:22:49.610 --> 00:22:52.730
OK, so this is the new definition of ILA.
00:22:54.900 --> 00:23:00.102
So an ILA is defined as a Chest CT
showing bilateral and nondependent ground
00:23:00.102 --> 00:23:04.089
glass opacities, reticular abnormalities,
lung distortion,
00:23:04.089 --> 00:23:08.886
traction bronchiectasis and or
honeycombing involving equal to or over
00:23:08.886 --> 00:23:12.738
5% of a lung zone.
And they define upper middle and lung
00:23:12.738 --> 00:23:16.860
zones by the inferior arch and right
inferior pulmonary vein
00:23:17.600 --> 00:23:21.467
for a total of 6 lung zones.
So this is very similar to the Fleischner
00:23:21.467 --> 00:23:25.390
Society paper. As mentioned,
the incidental portion has been taken out.
00:23:26.840 --> 00:23:30.291
They also talk about things that are not
considered ILAs,
00:23:30.291 --> 00:23:35.052
so they talk a little bit about extent.
So #1 if it's a mild focal abnormality,
00:23:35.052 --> 00:23:38.563
things like focal paraspinal fibrosis,
which we see a lot,
00:23:38.563 --> 00:23:41.718
you know that that's probably not
considered an ILA.
00:23:41.718 --> 00:23:46.122
They also talk about if something's
unilateral, it's probably not an ILA.
00:23:46.122 --> 00:23:50.645
The one caveat of that is if those
patients have a strong family history of
00:23:50.645 --> 00:23:54.870
familial pulmonary fibrosis,
there is some data to support that it can
00:23:54.870 --> 00:23:57.370
kind of start as a unilateral abnormality.
00:23:57.900 --> 00:24:00.663
And then finally, if it's really,
really extensive disease,
00:24:00.663 --> 00:24:03.103
which they go on to define, it's probably,
you know,
00:24:03.103 --> 00:24:06.650
should be considered interstitial lung
disease as opposed to an abnormality.
00:24:07.810 --> 00:24:11.358
The authors also talk about radiologic
patterns that aren't.
00:24:11.358 --> 00:24:15.256
Things like atelectasis, linear scarring,
non-emphysematous cysts,
00:24:15.256 --> 00:24:17.815
centrilobular nodularity.
PPFE importantly,
00:24:17.815 --> 00:24:22.294
is not considered as part of the ILA
criteria, heart failure and aspiration.
00:24:22.294 --> 00:24:26.774
And I think I showed in previous slides
part of this is because these things
00:24:26.774 --> 00:24:29.450
don't really predict the risk of
progression.
00:24:33.740 --> 00:24:38.464
The guidelines do keep the previous sub
categorizations of nonsubpleural,
00:24:38.464 --> 00:24:41.848
subpleural,
nonfibrotic and subpleural fibrotic with
00:24:41.848 --> 00:24:44.658
examples here on the left-hand side.
Again,
00:24:44.658 --> 00:24:49.510
the thought being that there is some
prognostic significance. So therefore,
00:24:49.510 --> 00:24:50.660
these were kept in
00:24:55.390 --> 00:25:00.358
and then interstitial lung abnormalities
and what's actually a definition of
00:25:00.358 --> 00:25:04.810
interstitial lung disease.
So I think everybody is really excited to
00:25:04.810 --> 00:25:10.165
to read this table trying to define kind
of where that spectrum line is for an ILA
00:25:10.165 --> 00:25:13.713
versus an ILD.
And so the criteria put forth had to do
00:25:13.713 --> 00:25:16.940
with symptoms, physiology,
imaging and pathology.
00:25:18.250 --> 00:25:21.890
So if somebody has symptoms,
the symptoms of interest being dyspnea
00:25:21.890 --> 00:25:26.173
and cough that a clinician can attribute
to interstitial lung disease or the CT
00:25:26.173 --> 00:25:30.349
scan, then it's probably a true disease.
There was comments about things like
00:25:30.349 --> 00:25:32.972
fatigue,
but really it was felt that dyspnea and
00:25:32.972 --> 00:25:36.559
cough again probably have the most
prognostic data behind them for
00:25:36.559 --> 00:25:40.950
interstitial lung disease and that's why
these were included as the main symptoms
00:25:42.630 --> 00:25:47.710
Physiology - So any abnormality in the
pulmonary function testing we're most
00:25:47.710 --> 00:25:51.867
interested in for ILD: FVC,
TLC or DLCO that the clinician can
00:25:51.867 --> 00:25:56.684
attribute to essentially the radiologic
pattern would qualify this as an
00:25:56.684 --> 00:25:58.400
interstitial lung disease.
00:25:59.120 --> 00:26:03.833
One thing important to note: that they
did actually make criteria about being
00:26:03.833 --> 00:26:08.486
defined as a value or Z-score below the
lower limit of normal. So, you know,
00:26:08.486 --> 00:26:12.837
when I went through training,
we were still using things like 80%, 75%.
00:26:12.837 --> 00:26:17.006
And so this has changed to Z-scores below
the lower limit of normal.
00:26:17.006 --> 00:26:21.961
The other piece that they put in here was
that if pulmonary function testing over
00:26:21.961 --> 00:26:26.795
time satisfies the physiologic criteria
for progressive pulmonary fibrosis that
00:26:26.795 --> 00:26:31.629
the clinician again can attribute to a
disease process. So many of us have seen
00:26:31.700 --> 00:26:34.970
patients that have "normal" pulmonary
function testing,
00:26:34.970 --> 00:26:39.116
but if you look back a year ago,
all of a sudden they had 200-300 more
00:26:39.116 --> 00:26:42.445
milliliters in FVC.
And so that's not really age-related
00:26:42.445 --> 00:26:45.424
changes.
And so I think that could be considered a
00:26:45.424 --> 00:26:46.300
disease process
00:26:49.110 --> 00:26:50.700
in terms of imaging.
00:26:52.180 --> 00:26:57.962
So the main take away is that if there is
fibrosis, it's abnormal. And again,
00:26:57.962 --> 00:27:04.263
fibrosis being defined by honeycombing or
reticulation with traction bronchiectasis.
00:27:04.263 --> 00:27:10.194
And they do give a cutoff that basically
is over 5% of the total lung volume by
00:27:10.194 --> 00:27:14.123
visual estimation. And so again,
a reminder for ILA,
00:27:14.123 --> 00:27:19.090
it's over 5% of a lung zone.
This is over 5% of total lung volume.
00:27:20.240 --> 00:27:22.815
Importantly,
if patients have serial CT scans,
00:27:22.815 --> 00:27:25.719
for example,
again like in lung cancer screening and
00:27:25.719 --> 00:27:29.335
you've got progressive fibrotic
abnormalities on serial CT scans,
00:27:29.335 --> 00:27:32.130
again that should be considered a disease
process.
00:27:32.820 --> 00:27:37.718
And then finally the presence of one of
the major fibrotic ILD patterns on chest
00:27:37.718 --> 00:27:37.900
CT.
00:27:38.730 --> 00:27:43.536
So those being usual interstitial
pneumonia, UIP, fibrotic HP,
00:27:43.536 --> 00:27:48.877
which if some of you have read the new
guidelines is now slowly being
00:27:48.877 --> 00:27:54.600
transitioned to bronchiolocentric
interstitial pneumonia or fibrotic NSIP.
00:27:55.310 --> 00:27:59.307
I think one of the things as radiologists
and clinicians we struggle with is that
00:27:59.307 --> 00:28:03.061
if there isn't that much disease extent,
it's harder to actually say whether
00:28:03.061 --> 00:28:07.107
there's a major fibrotic pattern or not.
But if there's certain features that kind
00:28:07.107 --> 00:28:10.130
of fit that criteria, again,
it's probably a disease process.
00:28:11.590 --> 00:28:15.949
So this again is just an example here
really highlighting some of the fibrotic
00:28:15.949 --> 00:28:19.316
changes that we would consider a disease
process. And again,
00:28:19.316 --> 00:28:23.290
I would quantify that visually,
there's over 5% of the total lung zone.
00:28:24.090 --> 00:28:27.577
Of note,
they did talk a lot about quantitative CT
00:28:27.577 --> 00:28:33.183
scan and I think there's a lot of value
and hopeful clinical applicability that's
00:28:33.183 --> 00:28:36.807
coming down the pipeline.
But at this point in time,
00:28:36.807 --> 00:28:42.140
quantitative CT was not part of the
definition in terms of visual assessments
00:28:42.140 --> 00:28:43.850
and volumes and follow up
00:28:45.500 --> 00:28:49.606
and then finally pathology.
So if there is pathology for whatever
00:28:49.606 --> 00:28:52.655
reason.
So I think realistically the most common
00:28:52.655 --> 00:28:57.322
scenario is somebody who's had a lung
cancer resected and sometimes in the
00:28:57.322 --> 00:29:02.300
adjacent parenchyma you see some major
fibrotic patterns such as UIP that could
00:29:02.300 --> 00:29:04.790
be considered a disease process as well.
00:29:05.570 --> 00:29:09.296
The guidelines go on to very clearly
state patients shouldn't have a lung
00:29:09.296 --> 00:29:12.116
sample for the sake of getting pathology.
But you know,
00:29:12.116 --> 00:29:15.894
if you have it for for another cause and
you've got one of these patterns,
00:29:15.894 --> 00:29:18.110
that's something that you need to
consider.
00:29:19.200 --> 00:29:19.900
OK,
00:29:21.090 --> 00:29:25.273
and then we'll go through this.
So this is the the kind of screening,
00:29:25.273 --> 00:29:29.157
detection recommendations,
the baseline assessment and follow up
00:29:29.157 --> 00:29:31.009
assessment that they put forth.
00:29:32.350 --> 00:29:35.780
Let's start off with the first box
talking about screening and detection.
00:29:36.660 --> 00:29:40.826
And so I mentioned that, you know,
this can be found incidentally,
00:29:40.826 --> 00:29:45.490
but there's certain populations that A,
you might go looking for it and B,
00:29:45.490 --> 00:29:50.092
might have CT scans for other reasons.
So the first one being lung cancer
00:29:50.092 --> 00:29:54.569
screening eligible populations.
If a patient is going to have a CT scan
00:29:54.569 --> 00:29:58.798
for lung cancer screening,
it should probably be evaluated for ILAs
00:29:58.798 --> 00:30:01.658
as well.
There's a lot of shared risk factors
00:30:01.658 --> 00:30:05.700
between lung cancer and ILD / ILAs,
things like smoking and age.
00:30:05.700 --> 00:30:08.809
And so I think that is slowly being
incorporated.
00:30:08.890 --> 00:30:13.231
A lot of the guidelines about incidental
findings in lung cancer screening
00:30:13.231 --> 00:30:15.836
includes something about pulmonary
fibrosis,
00:30:15.836 --> 00:30:18.383
maybe not specifically the terminology
ILA,
00:30:18.383 --> 00:30:22.320
but I think radiologist recognize they
might incidentally find that
00:30:23.810 --> 00:30:29.093
the next big one for screening was
patients diagnosed with connective tissue
00:30:29.093 --> 00:30:32.592
disease.
And so I think this is kind of keeping in
00:30:32.592 --> 00:30:38.288
line with the 2023 ACR chest guidelines.
This was a pretty big statement that came
00:30:38.288 --> 00:30:43.503
out in 2023-2024 that really made a
comment that patients with high risk of
00:30:43.503 --> 00:30:48.306
having ILD to probably have high
resolution CT scans as part of their
00:30:48.306 --> 00:30:51.668
initial evaluation for their autoimmune
disease,
00:30:51.668 --> 00:30:56.060
pulmonary function testing isn't enough,
we know that can be an
00:30:56.260 --> 00:30:59.857
insensitive marker for ILD.
And so this is really the first statement
00:30:59.857 --> 00:31:02.735
that says people with SARD,
if you're high risk of ILD,
00:31:02.735 --> 00:31:05.150
probably should get a high-resolution CT
scan.
00:31:05.150 --> 00:31:09.416
I think we've had a talk on this already
and I encourage you to read this document
00:31:09.416 --> 00:31:12.346
if you haven't.
They give a nice table on the right here
00:31:12.346 --> 00:31:15.943
about those most at risk for having
interstitial lung disease with an
00:31:15.943 --> 00:31:16.920
autoimmune disease.
00:31:20.190 --> 00:31:25.442
And then the next I think pretty bold
statement that the guidelines came out
00:31:25.442 --> 00:31:31.036
with was a screening recommendation for
first degree relatives over the age of 50
00:31:31.036 --> 00:31:34.720
who have a relative with familial
pulmonary fibrosis.
00:31:35.770 --> 00:31:40.627
And so I think this, you know,
really comes down to some of the data I
00:31:40.627 --> 00:31:45.622
presented earlier that having ILA's and
at risk family members is really
00:31:45.622 --> 00:31:47.880
prevalent, you know, one in four.
00:31:48.840 --> 00:31:52.611
So again, just to clarify,
this would be like if my dad has
00:31:52.611 --> 00:31:57.136
interstitial lung disease and my brother
has interstitial lung disease,
00:31:57.136 --> 00:32:01.598
I'm a first degree relative,
I should be screened at the age of 50 for
00:32:01.598 --> 00:32:02.289
these ILAs.
00:32:06.250 --> 00:32:09.820
they do talk about.
What about first degree relatives of
00:32:09.820 --> 00:32:14.580
those with idiopathic pulmonary fibrosis?
It sounds like this was a debated
00:32:16.140 --> 00:32:19.959
question. There was no consensus.
If you look at the numbers,
00:32:19.959 --> 00:32:24.457
the committee seemed 50/50 split.
Part of this is because if you look at
00:32:24.457 --> 00:32:28.461
the small available screening studies
that have happened so far,
00:32:28.461 --> 00:32:33.390
the numbers are actually pretty close.
Upwards of about 24% of patients who are
00:32:33.390 --> 00:32:38.380
first degree relatives of those with just
a single sporadic idiopathic pulmonary
00:32:38.380 --> 00:32:41.954
fibrosis case can have features of ILAs.
That being said,
00:32:41.954 --> 00:32:46.082
because the data was pretty small
compared to some of the familial
00:32:46.082 --> 00:32:48.299
pulmonary fibrosis screening cohorts
00:32:48.480 --> 00:32:50.970
that was not included and there was no
consensus.
00:32:52.210 --> 00:32:55.201
What's not considered familial pulmonary
fibrosis,
00:32:55.201 --> 00:32:59.600
sarcoidosis and PPFE aren't really
included in that definition of familial
00:32:59.600 --> 00:33:03.765
pulmonary fibrosis. In terms of the age,
I think it's a bit arbitrary,
00:33:03.765 --> 00:33:06.639
the 50 cut off of course again showed
some data.
00:33:06.639 --> 00:33:10.569
There's increased prevalence with age.
We have to start somewhere.
00:33:10.569 --> 00:33:14.440
They do comment though in some families
there might be, you know,
00:33:14.440 --> 00:33:19.132
a bit more penetrant gene that causes
earlier diagnosis and earlier patients to
00:33:19.132 --> 00:33:24.060
be affected and it would be reasonable to
screen five years before the 1st affected
00:33:24.620 --> 00:33:25.380
family member.
00:33:26.950 --> 00:33:31.907
The guidelines also comment that a lot of
these familial pulmonary fibrosis cohorts
00:33:31.907 --> 00:33:36.039
and genetic studies to date have been
from European ancestry cohorts.
00:33:36.039 --> 00:33:39.757
So I think in our, you know,
very diverse Canadian population,
00:33:39.757 --> 00:33:44.183
we have to take that - number one,
we need enriched cohorts that are a bit
00:33:44.183 --> 00:33:44.420
more
00:33:46.070 --> 00:33:49.673
multinational and just kind of make sure
that we're applying these to the right
00:33:49.673 --> 00:33:50.710
patient in front of us.
00:33:53.760 --> 00:33:56.950
OK, so you've identified ILAs.
Now what should we do?
00:33:58.290 --> 00:34:00.945
So of course,
you know it's appropriate to do a
00:34:00.945 --> 00:34:04.209
baseline assessment.
We want to look for those features of
00:34:04.209 --> 00:34:07.362
interstitial lung disease by doing a
symptom assessment,
00:34:07.362 --> 00:34:10.736
asking patients about cough,
exertional shortness of breath.
00:34:10.736 --> 00:34:15.328
We should probably ask them about things
like connective tissue disease and family
00:34:15.328 --> 00:34:18.149
history.
And then it's probably a good idea to get
00:34:18.149 --> 00:34:21.690
some pulmonary function testing again,
spirometry, TLC and DLCo
00:34:22.790 --> 00:34:27.485
it's worthwhile talking to patients about
risk reduction, recognizing that,
00:34:27.485 --> 00:34:30.451
you know,
the data supporting that this makes a
00:34:30.451 --> 00:34:33.788
really meaningful clinical outcome is is
forthcoming.
00:34:33.788 --> 00:34:38.607
But it's probably good to talk about
smoking cessation, exposure remediation,
00:34:38.607 --> 00:34:41.635
environmental,
occupation and medication and age
00:34:41.635 --> 00:34:43.180
appropriate vaccinations.
00:34:45.270 --> 00:34:48.338
Importantly,
recommendations against in terms of both
00:34:48.338 --> 00:34:52.828
for a screening purpose to decide who
should get a CT scan as well as once you
00:34:52.828 --> 00:34:56.749
have an ILA and determining, you know,
who's at risk of progression,
00:34:56.749 --> 00:35:01.465
the guideline recommends against testing
for the MUC5B as well as telomere length.
00:35:01.465 --> 00:35:05.671
I think both of these are really,
really exciting biomarkers in the field
00:35:05.671 --> 00:35:09.194
of interstitial lung disease.
They at some point they will be
00:35:09.194 --> 00:35:13.740
incorporated into baseline assessments,
but there's still a bit at the research
00:35:13.740 --> 00:35:14.650
phase right now.
00:35:15.910 --> 00:35:20.074
They also talk about lung sampling,
including bronchoalveolar lavage and
00:35:20.074 --> 00:35:23.782
transbronchial biopsies,
as well as cryo biopsies that's felt to
00:35:23.782 --> 00:35:28.290
be not necessary as a baseline assessment
for interstitial lung abnormalities.
00:35:31.570 --> 00:35:35.409
And then what about follow up assessment?
And so this is nice to see kind of a,
00:35:35.409 --> 00:35:37.665
you know,
a bit of numbers about how often you
00:35:37.665 --> 00:35:40.497
should be following these patients.
So of course, if they,
00:35:40.497 --> 00:35:43.904
you know on your first assessment you
determined that they've got ILD,
00:35:43.904 --> 00:35:46.688
they should go to the right,
they should be referred to a
00:35:46.688 --> 00:35:49.424
pulmonologist for evaluation,
management and monitoring.
00:35:49.424 --> 00:35:50.480
As for ILD guidelines,
00:35:51.310 --> 00:35:54.168
what about if you're really convinced
this is really just an ILA?
00:35:54.168 --> 00:35:56.420
How how long should be follow them,
How frequently?
00:35:57.210 --> 00:35:59.935
So of course, you know,
I like to tell my patients,
00:35:59.935 --> 00:36:03.813
monitor for signs of, you know,
progressive cough and shortness of breath
00:36:03.813 --> 00:36:05.910
and then repeat some PFT's and CT chest,
00:36:07.470 --> 00:36:10.895
they qualify ILAs as being either high
risk or low risk,
00:36:10.895 --> 00:36:15.823
with high risk suggesting PFTs every six
months to a year and imaging at the year
00:36:15.823 --> 00:36:20.450
mark and low risk being every two to
three years for both pulmonary function
00:36:20.450 --> 00:36:24.657
testing and imaging. And so,
what's considered high risk or low risk?
00:36:24.657 --> 00:36:27.241
Again,
it comes back to many of those data
00:36:27.241 --> 00:36:31.808
points I've already presented things like
demographic and clinical factors.
00:36:31.808 --> 00:36:35.594
If you do have a family history,
older age, genetic, you know,
00:36:35.594 --> 00:36:39.260
if for whatever reason you do have MUC5B
promoter variant or
00:36:39.340 --> 00:36:42.430
got telomere length testing on a patient.
And of course,
00:36:42.430 --> 00:36:46.931
the imaging characteristics are probably
I think kind of one of the biggest things
00:36:46.931 --> 00:36:51.378
if you do have signs of that fibrosis as
well as physiologic borderline pulmonary
00:36:51.378 --> 00:36:52.300
function testing.
00:36:55.030 --> 00:36:58.037
So you know,
there's no comments about you how long
00:36:58.037 --> 00:37:02.492
[forever] to follow them. Like I said,
the I think it really is by a case by
00:37:02.492 --> 00:37:05.326
case basis, but the guidelines do say,
you know,
00:37:05.326 --> 00:37:09.549
I think it's reasonable to at least do a
minimum of three to four years.
00:37:09.549 --> 00:37:14.234
Again reflecting on that Park et al data,
that if progression is going to happen
00:37:14.234 --> 00:37:15.970
the median time was 3.2 years.
00:37:20.050 --> 00:37:24.601
the guidelines go on to to give a lot of
work to to the community about unsolved
00:37:24.601 --> 00:37:27.243
questions and future research needs.
You know,
00:37:27.243 --> 00:37:31.570
I think that all of these are really,
really exciting lines of inquiry and I
00:37:31.570 --> 00:37:35.560
suspect we'll get some of these answers
over the next couple of years.
00:37:38.070 --> 00:37:40.380
So,
so that's the guidelines in a nutshell.
00:37:40.380 --> 00:37:44.530
Really looking forward to comments and
discussion. Just before I turn to that,
00:37:44.530 --> 00:37:48.626
I do want to take a minute just to talk
about maybe some of the challenges of
00:37:48.626 --> 00:37:50.990
implementing this in our Canadian
landscape.
00:37:51.710 --> 00:37:54.744
And I think everybody's busy to start off
with.
00:37:54.744 --> 00:37:59.170
We have a bit of a different family
practice setup of both, you know,
00:37:59.170 --> 00:38:02.711
locally here in Alberta as well as across
the province,
00:38:02.711 --> 00:38:05.683
many patients don't even have a family
doctor.
00:38:05.683 --> 00:38:08.654
And so who's going to be ordering these
tests?
00:38:08.654 --> 00:38:12.954
Who's going to be following up on these
tests and things like that?
00:38:12.954 --> 00:38:17.380
I think access to imaging here on the
left hand side is a huge issue.
00:38:18.680 --> 00:38:22.922
There are certainly ways that we could
expand access to imaging. Uh, you know,
00:38:22.922 --> 00:38:25.285
tele-radiology, mobile units,
purchase CTs.
00:38:25.285 --> 00:38:27.702
I know a lot here are a little bit
outdated,
00:38:27.702 --> 00:38:31.461
but of course that takes government input
and government funding and,
00:38:31.461 --> 00:38:34.200
and that's something that we need to
advocate for.
00:38:35.510 --> 00:38:39.566
I've mentioned workforce limitations,
both from a primary care perspective as
00:38:39.566 --> 00:38:43.831
well as from a respirology perspective.
And finally, as ILD physicians, you know,
00:38:43.831 --> 00:38:48.252
I think every province is struggling with
primary care recruitment. We've certainly,
00:38:48.252 --> 00:38:52.049
I'm sure been following some of the news
in certain provinces right now.
00:38:52.049 --> 00:38:56.313
So how do we make sure that to start off
with, family doctors actually, you know,
00:38:56.313 --> 00:39:00.318
are present to care for these patients
and refer them for simple things like
00:39:00.318 --> 00:39:04.739
lung cancer screening. And then of course,
we'd love to see some more ILD fellows go
00:39:04.739 --> 00:39:06.820
through to increase our, our practice of
00:39:06.900 --> 00:39:10.910
interstitial lung disease who many go on
to practice community general respirology
00:39:11.900 --> 00:39:15.694
in terms of socioeconomics,
I think that's important to address in
00:39:15.694 --> 00:39:18.979
Canada, you know,
it's a big country and a lot of care is
00:39:18.979 --> 00:39:22.547
often urban centric.
We know that in the world of interstitial
00:39:22.547 --> 00:39:25.662
lung disease,
patients often have to travel really far
00:39:25.662 --> 00:39:29.910
distances to get to subspecialty clinics.
And I know in Alberta, you know,
00:39:29.910 --> 00:39:33.818
the access to respirology really,
really varies where you are in the
00:39:33.818 --> 00:39:36.650
province.
And so how can we address this - things
00:39:36.650 --> 00:39:41.124
like providing travel subsidies as well
as, you know, public health campaigns,
00:39:41.124 --> 00:39:43.390
trying to recruit more respirologists to
00:39:43.650 --> 00:39:47.140
in need areas. And then finally,
of course, system level barriers.
00:39:48.580 --> 00:39:53.063
There are many different, you know,
networks across the the provinces as well
00:39:53.063 --> 00:39:56.800
as across the country.
Provinces don't really talk to each other
00:39:56.800 --> 00:40:01.111
when it comes to things like EMRs.
And so how do we make sure that there's
00:40:01.111 --> 00:40:04.675
an integrated care both locally,
provincially and nationally?
00:40:04.675 --> 00:40:08.067
Things like can we do care pathways and
referral networks?
00:40:08.067 --> 00:40:12.436
How do we incorporate some of these
recommendations into EMRs and of course
00:40:12.436 --> 00:40:15.195
promote CME both at the family physician
level,
00:40:15.195 --> 00:40:17.839
respirology level as well as radiology
level?
00:40:20.940 --> 00:40:26.051
So we'll come back to this poll question
and and this was developed a little bit
00:40:26.051 --> 00:40:30.343
before the guidelines came out.
And so based on the new guidelines,
00:40:30.343 --> 00:40:35.454
I would actually probably agree with 50%
of you that kind of C is the answer for
00:40:35.454 --> 00:40:40.440
who would actually qualify as kind of an
ILA based on these newest guidelines.
00:40:40.440 --> 00:40:44.290
And so these are all three patients of
mine that I care for.
00:40:45.230 --> 00:40:49.191
The first patient actually presented many,
many times for renal colic,
00:40:49.191 --> 00:40:52.260
always got a CT scan,
always had a little bit of these
00:40:52.260 --> 00:40:55.441
radiologic changes in the abdomen.
It was in the report,
00:40:55.441 --> 00:40:58.175
but wasn't really highlighted in the
impression.
00:40:58.175 --> 00:41:02.304
And you can see on each following CT scan
it was getting worse and worse.
00:41:02.304 --> 00:41:05.820
So this is just an example.
After two years of this happening,
00:41:05.820 --> 00:41:10.060
you can see clearly see some progression
with some traction bronchiectasis,
00:41:10.060 --> 00:41:13.408
ground glass changes.
Taking into account the difference in
00:41:13.408 --> 00:41:16.812
imaging techniques,
he had a really strong family history of
00:41:16.812 --> 00:41:16.980
ILD
00:41:17.150 --> 00:41:20.831
And when he met me, you know,
he was diagnosed with idiopathic
00:41:20.831 --> 00:41:24.454
pulmonary fibrosis.
Idiopathic pulmonary fibrosis is a really
00:41:24.454 --> 00:41:28.018
aggressive phenotype.
He was started on antifibrotics and is
00:41:28.018 --> 00:41:30.940
currently going undergoing transplant
evaluation.
00:41:32.940 --> 00:41:37.712
The second patient had a CT for nodule
follow up and then on a subsequent CT a
00:41:37.712 --> 00:41:41.276
few years later.
There was progression documented and they
00:41:41.276 --> 00:41:46.229
were referred even though they had normal
FVC and DLCo at the initial time of the
00:41:46.229 --> 00:41:49.794
nodule follow up.
By the time I met them they were already
00:41:49.794 --> 00:41:54.083
desaturating on their six-minute walk
test and required oxygen. Again,
00:41:54.083 --> 00:41:58.070
this patient was diagnosed with IPF and
started on antifibrotics.
00:41:58.990 --> 00:42:01.928
and then finally this female has been
asymptomatic,
00:42:01.928 --> 00:42:06.730
has had normal pulmonary function testing.
She's had a CT scan on follow up that you
00:42:06.730 --> 00:42:09.442
can see here.
A little bit of reticular change.
00:42:09.442 --> 00:42:13.285
It might not project well.
I'm planning on repeating CT scan in two
00:42:13.285 --> 00:42:17.579
years and if it's still stable I may
discharge her from my clinic with some
00:42:17.579 --> 00:42:20.800
recommendations about signs and symptoms
to monitor for.
00:42:23.570 --> 00:42:25.859
So in summary,
you know ILAs are prevalent.
00:42:25.859 --> 00:42:29.761
These referrals if you're already not
getting them are going to be coming.
00:42:29.761 --> 00:42:34.132
And so I think having some guidance about
what to do with them is really important.
00:42:34.132 --> 00:42:38.086
I think we are getting to the point that
an evidence based approach to risk
00:42:38.086 --> 00:42:39.960
stratify these patients is possible.
00:42:40.680 --> 00:42:44.864
Of course there's a huge healthcare
resource required to manage some of the
00:42:44.864 --> 00:42:49.435
expected volumes as CT scans become a bit
more ubiquitous. But I'm really excited.
00:42:49.435 --> 00:42:53.785
I think there's some really exciting
future biomarker and clinical trials that
00:42:53.785 --> 00:42:58.080
will give us even more tools to help
manage them, and it is a space to watch.
Learning objectives:
Describe the key updates in the 2025 ILA guidelines
Identify clinical scenarios where early recognition of ILA may impact patient outcomes and guide timely referral and investigation
Apply the 2025 ILA recommendations through case discussions to guide patient management
Program date: November 5, 2025
Speaker: Amanda Grant-Orser MBBCh FRCPC
Clinical Assistant Professor
Respiratory Medicine, Department of Medicine, University of Calgary
Director, Foothills Medical Centre Interstitial Lung Disease Clinic
FMC Health Sciences Centre
Moderator: Christopher Ryerson MD, FRCPC
Professor
Department of Medicine and Centre for Heart Lung Innovation
University of British Columbia
Questions?
The presenters are solely responsible for developing the content of this presentation and maintaining scientific accuracy, objectivity, and balance. Any discussions of clinical practice or treatment represent the opinions of the presenter alone. This video is made available for attendees, registrants, and individuals requesting further information on this learning program.