00:21.489 --> 00:25.290
IgG4 related disease is a rare chronic fiber
inflammatory disease that is characterized by
00:25.290 --> 00:29.290
unpredictable disease flares and can affect
nearly any organ in the body.
00:30.049 --> 00:35.490
Im Dr Emma Culver, a gastroenterologist
hepatologist at the University of Oxford United
00:35.490 --> 00:38.360
Kingdom. Where i will multidisciplinary team dedicated to patients with IgG4
00:38.360 --> 00:44.720
related disease, including gastrointestinal
manifestations
00:46.229 --> 00:53.220
such as Type autoimmune pancreatitis and IgG4 related
Colangitis Today Dr Calver and I are going to
00:53.220 --> 00:57.950
walk through some of the rational for a
multidisciplinary approach to IgG4 related
00:57.950 --> 01:03.389
disease and the importance of partnering across
specialties for patients living with IgG4 related
01:03.389 --> 01:06.260
disease.
to see beyond the symptoms and think
01:06.260 --> 01:13.260
systemically let's begin with a brief overview
of IgG4 related disease IgG4 related disease is
01:13.260 --> 01:16.480
a multiorgan disease that can appear in nearly
any organ.
01:17.860 --> 01:18.860
The organs affected 1988 related disease can vary
greatly across patients.
01:22.980 --> 01:26.459
The most commonly affected organs include the
lacrimal and salivary glands,
01:26.860 --> 01:32.620
the pancreas and the retroperitoneum, but many
others ranging from the orbits to the lungs and
01:32.620 --> 01:34.379
the aorta can also be impacted.
01:34.940 --> 01:39.900
The symptoms that patients present with depend
on the organ or organs involved.
01:40.500 --> 01:45.569
However, IgG4 related disease can also progress
over time to affect new organs.
01:46.540 --> 01:50.449
Dis Inflammation and fibrosis can lead to
structural and functional damage.
01:51.730 --> 01:57.169
Awareness of how structural damage and organ
impairment manifests is critical important to
01:57.169 --> 02:02.110
inform the timely diagnosis of IgG4 related
disease in your patients given the varied
02:02.110 --> 02:06.889
clinical presentation and often multi organ
involvement several specialists might be
02:06.889 --> 02:08.800
involved throughout the patient journey.
02:09.429 --> 02:14.229
Rheumatologists and gastroenterologists are
often the primary clinicians diagnosing and
02:14.229 --> 02:19.339
managing patients, although as we will discuss
shortly collaboration with radiologists and
02:19.339 --> 02:24.869
pathologists is crucial to make a diagnosis as
well as other specialists based on organ
02:24.869 --> 02:29.419
involvement diagnosis of IgG4 related disease may
be challenging.
02:30.009 --> 02:35.889
symptoms often overlap with other conditions,
including autoimmune and inflammatory disorders
02:35.889 --> 02:40.009
and malignancies.
There is no single fully reliable diagnostic
02:40.009 --> 02:46.770
marker diagnosis requires careful assessment of
clinical, radiologic serologic and
02:46.770 --> 02:48.369
histopathological features.
02:49.419 --> 02:54.259
Let's walk through some of these features in
more detail to determine when IgG4 related disease
02:54.259 --> 02:58.470
may be considered.
Generally speaking clinical and radiological
02:58.470 --> 03:05.229
features of IgG4 related disease include
diffuse or localized swellings strictures or a
03:05.229 --> 03:11.149
mass or nodule characteristic of IgG4 related
disease in my experience with gastrointestinal
03:11.149 --> 03:17.750
manifestations a IgG4 related disease such as IgG4
related autoimmune pancreatitis We often see
03:17.750 --> 03:21.619
diffuse pancreatic enlargement or focal masses
on imaging.
03:22.199 --> 03:24.940
In many cases patients also have biliary
structures IgG4 related cholangitis.
03:28.369 --> 03:34.389
These presentations can often mimic pancreatic
adenocarcinoma or cholangiocarcinoma and
03:34.389 --> 03:38.710
therefore clinicians need to be cautious to
exclude malignancies through additional
03:38.710 --> 03:45.630
assessments Emmanuel can you share typical
examples of characteristic imaging of IgG4 related
03:45.630 --> 03:51.139
disease sure presentation we see on imaging is
retroperitoneal fibrosis.
03:51.789 --> 03:57.350
As you can see here IgG4 related retropal
fibrosis can present on imaging as a soft
03:57.350 --> 03:59.580
tissue announcement surrounding the aorta.
04:00.350 --> 04:05.880
Assessing Sology and histopathology are also
crucial to distinguishing between IgG4 related
04:05.880 --> 04:07.470
disease and its mimickers.
04:07.830 --> 04:14.830
Thanks Emmanuel bring a great point lapworld to assess
serum I levels is an important component
04:14.830 --> 04:20.429
of a diagnostic worker for IgG4 related disease
as only twenty percent to forty percent of
04:20.429 --> 04:25.700
patients with IgG4 related disease present with a
normal baseline IGT level.
04:26.630 --> 04:32.070
However elevations in serum IT levels can occur
in other conditions and therefore assessing
04:32.070 --> 04:35.109
serology is just one of the many important
steps that are needed.
04:36.399 --> 04:43.119
pathological features of IgG4 related disease
included dense lymphocytic and IgG4 positive
04:43.119 --> 04:49.359
plasma cell infiltration and as you can see
here an increased ratio of IgG4 positive plasma
04:49.359 --> 04:52.390
cells to IgG4 positive plasma cells.
04:53.410 --> 05:00.399
Lastly typical tissue fibrosis such as a storm
fibrosis ohne Blistertip für weites can
05:00.399 --> 05:04.570
often be observed given the overlapping
clinical features and the importance of ruling
05:04.570 --> 05:09.649
out malignancies a multidisciplinary approach
to diagnosis and management can help clinicians
05:09.649 --> 05:10.910
feel confident in an IgG4 rate.
05:11.529 --> 05:17.089
Disease Diagnosis several organ specific
diagnostic criteria based on clinical
05:17.089 --> 05:21.239
radiologic serologic and pathologic features of
IgG4 related disease have been developed
05:24.529 --> 05:28.649
for instance gastroenterologists are likely
familiar with the His criteria for autoimmune
05:28.649 --> 05:33.660
pancreatitis.
organ specific criteria can help diagnose a
05:33.660 --> 05:39.019
single organ manifestation IgG4 related
disease is a multi-organ and progressive
05:39.019 --> 05:42.299
disease.
Therefore it is critical to evaluate patients
05:42.299 --> 05:47.450
holistically to understand the whole story of
the IgG4 related disease diagnosis.
05:48.250 --> 05:53.809
Additionally, the AR Euler classification
Criteria for IgG4 related disease provide a three
05:53.809 --> 05:59.160
step framework with high specificity and
sensitivity for classifying IgG4 related disease
05:59.690 --> 06:04.850
of note These classification criteria were
developed for clinical trial use and while they
06:04.850 --> 06:09.489
are a valuable tool in our toolbox says
clinicians they are not a diagnostic criteria.
06:10.299 --> 06:14.779
however the criteria provide clinicians a
valuable framework when considering the
06:14.779 --> 06:20.100
diagnosis of IgG4 related disease and may also
add in eliminating alternative diagnosis or
06:20.100 --> 06:22.019
mimikers of IgG4 related disease.
06:22.260 --> 06:28.140
The story of IgG4 related disease and patient
journey can be long and winding Patients might
06:28.140 --> 06:32.260
have disease that progresses to involve
additional organs before they finally receive a
06:32.260 --> 06:33.440
definitive diagnosis.
06:34.500 --> 06:39.000
It's important to add that the patient journey
does not end once the patients are diagnosed.
06:39.760 --> 06:44.130
This is a chronic disorder and most patients
will relapse after initial treatment.
06:45.040 --> 06:49.359
Monitoring is often advised to ensure patients
do not repeat the cycle of referrals and
06:49.359 --> 06:53.609
additional testing.
As a gastroenterologist it may be helpful to
06:53.609 --> 06:58.359
partner with a rheumatologist or continue to
monitor the disease outside your organ of
06:58.359 --> 07:01.959
specialty.
clinicians should consider an approach that
07:01.959 --> 07:06.720
works best for them with the understanding that
disease relapse may occur and that flare
07:06.720 --> 07:09.320
presentation can vary from initial diagnosis.
07:10.380 --> 07:14.980
clinical vigilance and Partnership with the
multidisciplinary team can help to optimize
07:14.980 --> 07:19.140
that patient's journey in summary today we
learned about the importance of a
07:19.140 --> 07:24.250
multidisciplinary approach to IgG4 related
disease and touched on the following key points.
07:25.549 --> 07:30.720
IgG4 related disease is a multi organ disease and
care coordination among specialists may help to
07:30.720 --> 07:34.640
optimize diagnosis and management to reduce
risk of disease flare.
07:35.540 --> 07:40.839
There is no single diagnostic test for IgG4
related disease clinicians have several tools
07:40.839 --> 07:47.320
available to help them confidently establish an
IgG4 related disease diagnosis disease
07:47.320 --> 07:52.799
awareness of disease characteristics and coordination specialists
help to improve patient experiences and
07:52.799 --> 07:54.420
outcomes. Thank you for listening to my colleague
Emma Calver discuss related disease today we are
08:03.570 --> 08:07.230
pleased to review this very important.
Please see other videos in this series from my
08:07.230 --> 08:10.329
colleagues for more information on IgG4
related disease,
08:10.750 --> 08:16.549
including Disease Overview Pathophysiology and
proactive Management of IgG4 related
08:16.549 --> 08:17.130
disease.
