Recognition of Pulmonary Fibrosis in the Primary Care Setting: Prompt Assessment and Timely Referral to Respirology

00:00:00.060 --> 00:00:03.076
Thank you for the opportunity to speak to
you today.

00:00:03.076 --> 00:00:07.515
Today I'm talking to you about recognition of
Pulmonary Fibrosis in the primary care

00:00:07.515 --> 00:00:10.360
setting, prompt assessment and timely referral to

00:00:10.360 --> 00:00:14.515
respirology. I'm Doctor Sacha Bhinder,
an adult respirologist in Toronto,

00:00:14.515 --> 00:00:16.450
ON.Sharing with you my disclosures

00:00:17.090 --> 00:00:19.634
for reference, our disclaimer for mitigation of

00:00:19.634 --> 00:00:23.451
potential bias and recognition of the
generous in kind support from our

00:00:23.451 --> 00:00:27.110
colleagues of Boehringer Ingelheim in the
form of logistical support.

00:00:28.150 --> 00:00:32.457
I have three primary objectives today.
The 1st is looking at key pearls for

00:00:32.457 --> 00:00:36.595
early recognition of Interstitial Lung
Disease revolving around history,

00:00:36.595 --> 00:00:39.202
symptoms, physical exam and imaging findings.

00:00:39.202 --> 00:00:43.510
The second is a review of the broad
differential diagnosis of ILD that your

00:00:43.510 --> 00:00:46.061
friendly neighborhood respirologist will
do.

00:00:46.061 --> 00:00:50.595
But mainly the third is the benefit of
early recognition of ILD by primary care

00:00:50.595 --> 00:00:54.450
and the role of collaboration between
primary care and respirology.

00:00:55.700 --> 00:00:59.643
We're going to start with a case,
but I'm actually demonstrating the case

00:00:59.643 --> 00:01:03.321
as I first saw the patient in the
emergency department. This was CW.

00:01:03.321 --> 00:01:07.531
He presented to the emergency department.
He had had a history of profound and

00:01:07.531 --> 00:01:11.422
marked deterioration and dyspnea,
exertional capacity and the ability to

00:01:11.422 --> 00:01:14.939
remain at home in this state.
He had worsening dyspnea and central

00:01:14.939 --> 00:01:17.551
chest discomfort on exertion.
He was frustrated.

00:01:17.551 --> 00:01:19.469
He went to the emergency department.

00:01:20.160 --> 00:01:22.960
He was hypoxemic,
walking to triage. His chest

00:01:22.960 --> 00:01:26.178
X-ray noted nonspecific peripheral basilar markings.

00:01:26.178 --> 00:01:30.588
He had a CT scan of the chest to rule out
pulmonary embolism based on his

00:01:30.588 --> 00:01:35.416
presentation by our emergency department
colleague. Reassuringly, no sign of PE,

00:01:35.416 --> 00:01:39.289
but it demonstrated Interstitial Lung
Disease with honeycombing.

00:01:39.289 --> 00:01:42.805
Given his symptoms,
he was admitted under general medicine

00:01:42.805 --> 00:01:46.262
for a possible ILD flare but also a new
diagnosis of ILD.

00:01:46.262 --> 00:01:50.433
He was seen by respirology myself.
We prescribe oxygen for exertional

00:01:50.433 --> 00:01:50.970
hypoxemia

00:01:51.090 --> 00:01:53.774
and referred him for ongoing outpatient
care.

00:01:53.774 --> 00:01:56.050
But let's see how we got to this point.

00:01:56.950 --> 00:02:01.169
CW's journey to the emergency department
actually started a year prior.

00:02:01.169 --> 00:02:04.919
Comorbidities consist of hypertension and
hypercholesterolemia.

00:02:04.919 --> 00:02:08.142
15 pack year tobacco history having quit
10 years ago.

00:02:08.142 --> 00:02:12.830
Hypercholesterolemia managed with Crestor
rosuvastatin and hypertension managed

00:02:12.830 --> 00:02:16.463
with Norvasc amlodipine.
He's a retired film and movie camera

00:02:16.463 --> 00:02:19.451
operator. He put 30 years of work into a unionized

00:02:19.451 --> 00:02:21.854
role. He retired and a year prior to the

00:02:21.854 --> 00:02:25.253
emergency department,
his journey of respiratory symptoms

00:02:25.253 --> 00:02:27.480
started. He presented with progressive

00:02:27.550 --> 00:02:31.566
dyspnea on exertion for six months.
His ECG was performed as within normal

00:02:31.566 --> 00:02:33.976
limits. He retired about a year prior and he

00:02:33.976 --> 00:02:37.082
admits he was deconditioned.
He worked hard for 30 years.

00:02:37.082 --> 00:02:40.242
He let himself go when he retired,
gained a bit of weight,

00:02:40.242 --> 00:02:42.919
obviously wasn't doing as much physical
activity.

00:02:42.919 --> 00:02:46.989
His primary care practitioner noted
basilar crackles on exam but given some

00:02:46.989 --> 00:02:50.310
abdominal adiposity,
thought this was related to Atelectasis.

00:02:50.990 --> 00:02:54.444
He wasn't clubbed and he had a normal
oxygen saturation at rest.

00:02:54.444 --> 00:02:57.420
The plan at that visit was to exercise
and lose weight.

00:02:58.410 --> 00:03:02.024
Crackles are pathologic.
When you hear crackles,

00:03:02.024 --> 00:03:06.672
it raises concern of parenchymal
interstitial disease and ILD.

00:03:06.672 --> 00:03:11.024
Clubbing is a late sign.
Resting hypoxemia is a late sign.

00:03:11.024 --> 00:03:14.786
Something was going on with CW even at
that visit,

00:03:14.786 --> 00:03:17.590
and the crackles were the key finding.

00:03:18.780 --> 00:03:19.680
But we persisted.

00:03:20.360 --> 00:03:24.090
After three months, he came back.
He saw his primary care practitioner.

00:03:24.090 --> 00:03:26.402
Repeat ECG was again within normal limits.

00:03:26.402 --> 00:03:30.553
He didn't have any meaningful improvement
with diet, exercise and weight loss.

00:03:30.553 --> 00:03:33.706
He was referred for a stress
echocardiogram. It was normal,

00:03:33.706 --> 00:03:37.700
but it showed reduced exercise capacity.
But his left ventricle was normal.

00:03:38.460 --> 00:03:43.047
He hit a maximum heart rate of 110%
predicted. It is uncommon for patients

00:03:43.047 --> 00:03:47.138
with cardiac disease to hit maximum heart
rate of 110% predicted.

00:03:47.138 --> 00:03:51.849
His heart was screaming on the stress
test trying to pump blood and pump as

00:03:51.849 --> 00:03:56.808
hard as possible because it didn't know
why his body couldn't do what he wanted

00:03:56.808 --> 00:03:57.180
to do.

00:03:58.050 --> 00:04:02.660
His oxygen saturation was not measured.
In fact oxygen testing is usually not

00:04:02.660 --> 00:04:07.152
performed for cardiac stress test.
That maximum heart rate of 100 and 110%

00:04:07.152 --> 00:04:10.403
predicted is a sign something non cardiac
is going on.

00:04:10.403 --> 00:04:13.832
So at this point he has non specific
symptoms of dyspnea,

00:04:13.832 --> 00:04:17.496
exertional intolerance with a normal ECG,
normal stress test,

00:04:17.496 --> 00:04:21.280
no meaningful improvement with diet,
lifestyle and weight loss.

00:04:22.730 --> 00:04:27.506
Another three months go by and now he
still has progressive dyspnea on exertion,

00:04:27.506 --> 00:04:31.693
but he has a progressive dry cough.
The cough is noted in the daytime,

00:04:31.693 --> 00:04:35.880
the cough is noted at night.
It is a dry cough with absence of sputum.

00:04:36.780 --> 00:04:41.021
He's given a diagnosis of COPD clinically
as well as chronic bronchitis.

00:04:41.021 --> 00:04:45.496
Though I will caution there was no sputum
and he's prescribed to you tiotropium

00:04:45.496 --> 00:04:48.924
spiriva. Unfortunately,
pulmonary function testing was not

00:04:48.924 --> 00:04:51.713
performed.There was no benefit with tiotropium

00:04:51.713 --> 00:04:55.084
after a couple of weeks,
but he was a good patient and he

00:04:55.084 --> 00:04:56.130
continued with it.

00:04:57.380 --> 00:05:00.689
He's given a clinical diagnosis of COPD.
Remember,

00:05:00.689 --> 00:05:05.686
emphysema is a radiographic diagnosis on
CT. Chronic bronchitis is a clinical

00:05:05.686 --> 00:05:10.748
diagnosis, but COPD requires spirometry.
You can only diagnose COPD with lung

00:05:10.748 --> 00:05:14.383
function testing,
and he did not have an opportunity to

00:05:14.383 --> 00:05:18.341
have confirmatory testing.
He was given a diagnosis based on

00:05:18.341 --> 00:05:22.235
nonspecific symptoms and there was no
benefit with Spiriva,

00:05:22.235 --> 00:05:25.740
which is also a sign that something else
is going on.

00:05:26.640 --> 00:05:32.539
That takes us to the emergency department
visit where frustrated, dyspneic, hypoxemic

00:05:32.539 --> 00:05:36.021
on exertion.
We have a diagnosis of interstitial

00:05:36.021 --> 00:05:40.712
markings on chest X-ray,
confirmatory diagnosis on CT chest and a

00:05:40.712 --> 00:05:43.840
new diagnosis of Interstitial Lung
Disease.

00:05:45.550 --> 00:05:50.065
There is a broad differential for
fibrotic Interstitial Lung Disease.

00:05:50.065 --> 00:05:55.354
Environmental and occupational factors
such as asbestos, silicosis, birds, molds,

00:05:55.354 --> 00:05:59.353
environmental allergens can cause ILD.
Radiation to the lung,

00:05:59.353 --> 00:06:03.417
radiation to the breast,
also reflecting on the lung can cause

00:06:03.417 --> 00:06:06.900
interstitial changes as can drugs.
3 common culprits,

00:06:06.900 --> 00:06:09.738
Amiodarone prescribed by our
cardiologists,

00:06:09.738 --> 00:06:14.511
Macrobid Nitrofurantoin for urinary tract
infections and Methotrexate for

00:06:14.511 --> 00:06:16.639
management of autoimmune disease.

00:06:16.790 --> 00:06:20.752
And of course, at the top right,
a whole host of autoimmune conditions can

00:06:20.752 --> 00:06:24.081
lead to Interstitial Lung Disease,
as can atypical infections,

00:06:24.081 --> 00:06:27.780
COVID infection and gastroesophageal
reflux. But what we worry about,

00:06:28.510 --> 00:06:32.181
what we often see,
is this idiopathic Pulmonary Fibrosis,

00:06:32.181 --> 00:06:37.245
once we've excluded all known causes,
left with an element of Interstitial Lung

00:06:37.245 --> 00:06:40.980
Disease for which we do not have a clear
pathologic cause.

00:06:42.280 --> 00:06:47.371
How do you explain this to the patient?
You explain that fibrosing Interstitial

00:06:47.371 --> 00:06:50.426
Lung Diseases,
a serious lung disease where the

00:06:50.426 --> 00:06:54.436
interstitial tissue,
the sponge of the lung, becomes inflamed,

00:06:54.436 --> 00:06:59.401
damaged, thickened, stiff and scarred.
It is a progressive fibrotic condition

00:06:59.401 --> 00:07:03.920
that continuously worsens over time.
Give it time, it will deteriorate.

00:07:04.680 --> 00:07:08.340
On the right hand side,
most patients with ILD are cared for by

00:07:08.340 --> 00:07:12.287
respirology and co-managed with other
specialists like rheumatology

00:07:12.287 --> 00:07:16.634
if we suspect connective tissue disease.
We regularly monitor patients with

00:07:16.634 --> 00:07:20.696
fibrosing ILD at regular intervals to
assess stability and monitor for

00:07:20.696 --> 00:07:23.270
progression.
Because while there is no cure,

00:07:23.270 --> 00:07:27.960
there are treatments available that can
slow the rate of decline of lung function

00:07:27.960 --> 00:07:32.708
and keep lung function at a higher level
for the future than without pharmacologic

00:07:32.708 --> 00:07:33.280
treatment.

00:07:34.600 --> 00:07:39.842
The signs and symptoms of fibrosing ILD
though are nonspecific. And remember,

00:07:39.842 --> 00:07:43.674
these are older patients,
so the onset can be insidious.

00:07:43.674 --> 00:07:47.101
Dyspnea is often insidious and slowly
progressive,

00:07:47.101 --> 00:07:52.344
but the dry hacking cough is commonly
seen in ILD. Patients with COPD tend to

00:07:52.344 --> 00:07:55.571
have chronic bronchitis and a productive
cough.

00:07:55.571 --> 00:08:01.015
Patients with ILD tend to have that dry
hacking day and night cough and they have

00:08:01.015 --> 00:08:05.250
crackles. Do not ignore crackles.
If you send a referral to me

00:08:05.340 --> 00:08:09.357
saying I auscultated crackles,
I'm worried about Interstitial Lung

00:08:09.357 --> 00:08:12.295
Disease.That is all I need to hear because what

00:08:12.295 --> 00:08:16.372
you heard shares my concern that this
patient has fibrosis of their

00:08:16.372 --> 00:08:21.289
interstitium of the sponge of the lung.
And I'm going to investigate for ILD and

00:08:21.289 --> 00:08:26.206
I want to do this early because clubbing
is often a late sign. Fatigue, lethargy,

00:08:26.206 --> 00:08:30.643
generally feeling unwell is a progressive
finding of progressive disease.

00:08:30.643 --> 00:08:33.761
And remember most patients are overweight
or obese.

00:08:33.761 --> 00:08:35.860
This is unfortunately quite common.

00:08:36.020 --> 00:08:40.439
When they start losing weight with ILD,
they might actually feel a bit better.

00:08:40.439 --> 00:08:44.802
It's only when they are a normal weight
and threatening to become underweight

00:08:44.802 --> 00:08:49.110
that this degree of weight loss becomes a
concern and they start saying, OK,

00:08:49.110 --> 00:08:51.851
now I'm really losing weight,
this is a problem.

00:08:51.851 --> 00:08:56.159
It may be late in the journey for that
patient that they are concerned about

00:08:56.159 --> 00:08:59.515
their weight loss.
Symptoms of cough and breathlessness are

00:08:59.515 --> 00:09:03.039
nonspecific and may perceive the
diagnosis by up to two years.

00:09:04.520 --> 00:09:09.256
Fibrosing ILD diagnosis is often delayed.
Access to diagnostic testing is a

00:09:09.256 --> 00:09:12.435
challenge.No standardized process is a challenge.

00:09:12.435 --> 00:09:17.359
But half of patients will tell us that over a year has passed before they were

00:09:17.359 --> 00:09:21.660
correctly diagnosed with ILD.
And the reason why we're here today is

00:09:21.660 --> 00:09:26.584
that colleagues in primary care play a
key role in improving that knowledge of

00:09:26.584 --> 00:09:31.321
ILD with education and awareness to
facilitate early diagnosis and referral.

00:09:31.321 --> 00:09:35.310
Because delay in diagnosis has consequences and we can quantify

00:09:35.380 --> 00:09:39.906
that. Delays in early referral are
associated with a poor prognosis.

00:09:39.906 --> 00:09:43.235
The X axis here is years,
the Y axis is survival,

00:09:43.235 --> 00:09:48.694
and the longer you unfortunately have to
wait before you have a diagnosis of ILD,

00:09:48.694 --> 00:09:53.287
the greater the likelihood that you will
not be here in this world.

00:09:53.287 --> 00:09:58.880
Delays lead to death and delays lead to a
poor prognosis for our patients with ILD.

00:10:00.410 --> 00:10:05.561
The diagnostic process for fibrosing ILD
involving respirology is a comprehensive

00:10:05.561 --> 00:10:09.518
history and physical exam building upon
your referral package,

00:10:09.518 --> 00:10:14.732
generating a broad differential diagnosis
in the categories that we had discussed.

00:10:14.732 --> 00:10:18.753
Additional tests such as connective
tissue disease blood work,

00:10:18.753 --> 00:10:23.464
pulmonary function testing, CT scan,
and developing a working diagnosis of

00:10:23.464 --> 00:10:27.987
what we think is causing the ILD and if
we've excluded all known causes,

00:10:27.987 --> 00:10:30.940
we're left with idiopathic Pulmonary
Fibrosis.

00:10:32.140 --> 00:10:36.226
Radiologic assessment is crucial please
when you hear crackles.

00:10:36.226 --> 00:10:39.674
When a patient has non specific
respiratory symptoms,

00:10:39.674 --> 00:10:42.483
assess lung structure,
order a chest X-ray.

00:10:42.483 --> 00:10:47.527
A chest X-ray is generally considered non
diagnostic because it's not specific

00:10:47.527 --> 00:10:50.784
enough to assess for ILD with fine
resolution,

00:10:50.784 --> 00:10:54.360
but it is helpful to alert us to an
underlying process.

00:10:55.050 --> 00:10:59.284
A patient with crackles,
a patient with non specific symptoms and

00:10:59.284 --> 00:11:02.235
a normal chest X-ray,
please order a CT scan.

00:11:02.235 --> 00:11:06.726
A normal chest X-ray with ongoing
symptoms should be followed up with

00:11:06.726 --> 00:11:11.537
further imaging because a CT scan can
accurately characterize and diagnose

00:11:11.537 --> 00:11:15.964
Interstitial Lung Disease
and we use it serially to assess response

00:11:15.964 --> 00:11:16.670
to therapy.

00:11:18.010 --> 00:11:22.363
From a primary care perspective,
the red flags are this progressive,

00:11:22.363 --> 00:11:26.023
insidious dyspnea,
a dry cough that occasionally might be

00:11:26.023 --> 00:11:30.124
productive but really is dry,
and crackles. These are insidious,

00:11:30.124 --> 00:11:33.846
progressive symptoms.
And I know that you will do your due

00:11:33.846 --> 00:11:37.884
diligence and try to treat possible
causes of these conditions.

00:11:37.884 --> 00:11:40.913
And if a patient fails to respond to
treatment,

00:11:40.913 --> 00:11:44.635
fails to respond to empiric therapy like
CW, it showed us,

00:11:44.635 --> 00:11:47.980
alert us to something else that's going
on. Crackles

00:11:48.210 --> 00:11:51.821
should not be ignored. Clubbing, remember,
is a late sign.

00:11:51.821 --> 00:11:55.923
And many of the nonspecific
constitutional complaints, patients can

00:11:55.923 --> 00:12:01.066
justify and rationalize to something else.
If the chest X-ray suggests interstitial

00:12:01.066 --> 00:12:05.657
changes, please order a CT scan.
If the chest X-ray is normal and you have

00:12:05.657 --> 00:12:09.269
nonspecific symptoms and crackles,
please order a CT scan.

00:12:09.269 --> 00:12:12.330
If you have access to pulmonary function
testing,

00:12:12.330 --> 00:12:16.248
you will often see reduced lung volumes
and abnormal diffusion.

00:12:16.248 --> 00:12:18.329
And the advantage of the pandemic,

00:12:18.480 --> 00:12:22.317
patients are ordering and using pulse
oximeters to monitor saturation.

00:12:22.317 --> 00:12:24.696
They may notice desaturation with
exertion.

00:12:24.696 --> 00:12:27.398
You may have a resting saturation in your
office,

00:12:27.398 --> 00:12:30.750
but the patient will tell you what's
happening with exertion.

00:12:31.490 --> 00:12:34.535
The role of primary care here is early
recognition,

00:12:34.535 --> 00:12:39.395
assessment of exposures and medications,
feeding that information to your friendly

00:12:39.395 --> 00:12:43.260
neighborhood respirologist,
and set the ball rolling with initial

00:12:43.260 --> 00:12:47.067
investigations of chest X-ray, PFT,
exclusion of cardiac causes.

00:12:47.067 --> 00:12:49.702
A CT scan is nice to have,
but we can do it.

00:12:49.702 --> 00:12:53.625
And if you have challenges accessing PFT,
stress testing, CT scan,

00:12:53.625 --> 00:12:58.310
your friendly neighborhood respirologist
often has a way of getting these tests

00:12:58.310 --> 00:13:02.820
sooner. But please refer to respirology,
inform them you're concerned about

00:13:02.890 --> 00:13:06.403
ILD. Tell them why and we will listen,
we will investigate,

00:13:06.403 --> 00:13:10.865
we will pursue and we will include or
exclude the possibility that this patient

00:13:10.865 --> 00:13:13.543
has ILD.
We will then of course co-manage these

00:13:13.543 --> 00:13:17.280
patients with you because this is a
disease that does not go away.

00:13:18.420 --> 00:13:23.232
What's nice to have in a referral, symptom
list and duration, what you've heard,

00:13:23.232 --> 00:13:27.503
what the patient has told you,
what you've tried and what you've done.

00:13:27.503 --> 00:13:32.256
Crackles on auscultation or any clubbing,
history of exposures to allergens and

00:13:32.256 --> 00:13:37.129
occupational history, testing as we've
discussed as well. These are nice to have,

00:13:37.129 --> 00:13:40.377
not a need to have.
Your concern for ILD, crackles on

00:13:40.377 --> 00:13:44.949
auscultation is enough for me to say I'm
going to keep the ball rolling and

00:13:44.949 --> 00:13:48.859
continue to investigate and dig deeper.
Primary care colleagues,

00:13:48.950 --> 00:13:53.114
have a meaningful opportunity to identify
these patients with ILD in a timely

00:13:53.114 --> 00:13:57.507
manner and refer for specialty care.
We don't see patients without referral,

00:13:57.507 --> 00:14:00.531
unfortunately.
We need your eyes and ears and taking

00:14:00.531 --> 00:14:03.156
that history to capture these patients
early.

00:14:03.156 --> 00:14:07.948
Because this is a progressive condition,
we need to have a high index of suspicion.

00:14:07.948 --> 00:14:12.113
We need early diagnosis and referral.
I have 3 pearls to leave you with.

00:14:12.113 --> 00:14:15.593
This typically presents with dyspnea on
exertion, dry cough,

00:14:15.593 --> 00:14:19.530
and a change in clinical activity and
exercise that can be insidious

00:14:19.620 --> 00:14:23.880
and the patient and us can incorrectly
attribute it to something else.

00:14:23.880 --> 00:14:26.580
Crackle should not be ignored.
At a minimum,

00:14:26.580 --> 00:14:31.020
chest imaging should be ordered and if
crackles persist, X-ray is normal,

00:14:31.020 --> 00:14:34.020
please order a CT scan.
If the X-ray is abnormal,

00:14:34.020 --> 00:14:37.800
please order a CT scan.
If you can't get a CT scan in a timely

00:14:37.800 --> 00:14:40.500
manner, tell us and we will happily order it

00:14:40.500 --> 00:14:43.200
because that is our role and
responsibility.

00:14:44.030 --> 00:14:48.917
COPD is not a clinical diagnosis.
You need lung function testing at a

00:14:48.917 --> 00:14:53.804
minimum spirometry to diagnose COPD.
Chronic bronchitis is a clinical

00:14:53.804 --> 00:14:57.434
diagnosis. Restrictive Physiology is what we see on

00:14:57.434 --> 00:15:02.112
a PFT in the setting of ILD.
COPD and ILD can coexist. But please,

00:15:02.112 --> 00:15:07.488
if you're going to make a diagnosis of
COPD, if you have clinical suspicion,

00:15:07.488 --> 00:15:12.584
order a pulmonary function test because
that is how we diagnose COPD and

00:15:12.584 --> 00:15:14.400
recognize that restrictive

00:15:14.490 --> 00:15:18.010
physiology is what we typically see on a
PFT in the setting of ILD.

00:15:18.010 --> 00:15:20.805
It has been a privilege to share this
with you today.

00:15:20.805 --> 00:15:23.290
Thank you for your participation and
attention.