00:27.860 --> 00:29.290
I'm also the founder and executive.
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IgG4 Forward Foundation, the first organisation
dedicated specifically
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to IgG4 related disease advocacy research and
support today I'm going to discuss what IgG4
00:42.582 --> 00:48.841
related disease is, including its clinical
presentation and typical disease course I will
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also elaborate on how our understanding of IgG4
related. As a systemic
00:55.554 --> 00:57.104
condition recently emerged.
00:57.344 --> 01:00.394
IgG4 related disease is not a new disease.
01:00.714 --> 01:07.234
Its first recognition in the medical literature
appears to date back to 1892 the description of a
01:07.234 --> 01:14.234
forty two year old man a farmer who had
painless bilateral swelling of lacrymal glands
01:14.234 --> 01:16.194
and his major salary glands. However
01:16.825 --> 01:21.816
understanding of it as a systemic disease did
not occur until 2003. When we
01:25.066 --> 01:29.646
researchers from Japan described autoimmune
pancreatitis as part of a larger systemic
01:29.646 --> 01:36.566
disease that we now know as IgG4 related disease
over the past two decades our understanding
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of IgG4 related disease has evolved
significantly in 2011.
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IgG4 related disease was recognized at the First
International Symposium on IgG4 related Disease
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in 2019 Classification Criteria for IgG4 related
disease were established.
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now let's move on to what we know today in terms of
the epidemiology of IgG4 related disease.
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IgG4 related disease can affect both men and women
tends to have a male predominance.
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The typical age diagnosis is fifty to seventy
years but IgG4 related disease can appear
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in individuals of essentially any estimating
the total impact of IgG4 related disease is
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challenging since the epidemiologic data are
limited.
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Recent data from the US show that IgG4 related
disease has an incidence rate of approximately
02:38.520 --> 02:40.940
one per 100 thousand individuals and a
prevalence of approximately five per 100
02:40.940 --> 02:42.619
thousand individuals IgG4 related diseases.
02:44.134 --> 02:49.535
therefore has a similar incidence and
prevalence as other rheumatic diseases such as
02:49.895 --> 02:53.134
associated vasculitis and systemic sclerosis.
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Fortunately we are continuing to learn more
each day through research and growing awareness
02:59.074 --> 03:05.500
in the medical community.
so what exactly is IgG4 related disease IgG4
03:05.500 --> 03:10.619
related disease is a chronic systemic disorder
that is characterized by tumor inflammatory
03:10.619 --> 03:17.339
masses and tissue fibrosis IgG4 related disease
can present in
03:17.339 --> 03:23.100
nearly any organ and frequently affects
multiple organs simultaneously by the time it's
03:23.100 --> 03:28.809
diagnosed at the time of diagnosis.
patients an average of three affected organs
03:37.910 --> 03:38.940
and over the course of the disease this may
progress to include additional organs.
03:38.960 --> 03:39.229
Hallmarks of the disease include an elevated
serum IgG4 concentration,
03:39.240 --> 03:45.509
increased numbers of IgG4 positive plasma cells in
tissue and circulating plasma blasts.
03:45.710 --> 03:49.740
Most patients have elevated serum IgG4 levels at
baseline.
03:50.330 --> 03:56.759
20 to forty percent have normal values and this
does not rule out the possibility that IgG4
03:56.759 --> 03:58.669
related disease might be present.
03:58.919 --> 04:04.639
The diagnosis of IgG4 related disease is
challenging and may lead to a prolonged patient
04:04.639 --> 04:08.789
journey with misdiagnoses of cancer not
uncommon.
04:09.009 --> 04:15.529
The clinical presentation of IgG4 related disease
is heterogeneous and it can progress undetected.
04:16.470 --> 04:22.959
It can occur in nearly any organ, but some organs are
more frequently affected such as the pancreas
04:23.329 --> 04:28.929
bile ducts, retroperitoneum, lacrymal glands
and major salivary glands.
04:29.209 --> 04:32.359
The pancreas is one of the most frequently
impacted organs.
04:32.779 --> 04:38.369
It is affected in approximately forty five
percent of patients and is often accompanied by
04:38.369 --> 04:43.059
biliary strictures patients with IgG4 related
disease also occasionally.
04:44.114 --> 04:50.904
disease affecting the orbits lungs or kidneys
as you see on the image other organs can also
04:50.904 --> 04:57.545
be involved but may be less common overall it
is important to know that IgG4 related disease can
04:57.545 --> 05:04.225
occur virtually anywhere in the body which make
ongoing management and monitoring challenging a
05:04.225 --> 05:09.864
focus on addressing the immediate symptoms of IgG4
related disease as opposed to chronic
05:09.864 --> 05:14.380
management could
contribute to unpredictable flares and disease
05:14.380 --> 05:19.929
progression because of the insidious slow
moving nature of IgG4 related disease
05:20.100 --> 05:25.420
approximately sixty percent of patients have
irreversible organ damage by the time they are
05:25.420 --> 05:30.720
diagnosed awareness of how organ damage
manifests itself is critically important.
05:31.149 --> 05:38.109
to informing the timely diagnosis of IgG4 related
disease Disease recurrences often cause damage
05:38.109 --> 05:43.299
in new sites of disease or in organs previously
affected by the condition.
05:43.309 --> 05:48.350
Patients may have a higher chance of relapse if
they have risk factors such as multi organ
05:48.350 --> 05:55.130
involvement or elevated IgG4 IGE or blood eosinophil
counts at
05:55.130 --> 05:59.429
diagnosis.
in some instances relapses may occur when
05:59.429 --> 06:03.720
tapering or after withdrawing glucocorticoid
therapy.
06:04.239 --> 06:10.230
with the unpredictable nature of IgG4 related
disease it's difficult to determine and when it
06:10.230 --> 06:12.820
will advance to irreversible disease.
06:13.029 --> 06:17.390
Patients can experience a variety of
manifestations related to the functional and
06:17.390 --> 06:21.470
structural damage associated with IgG4 related
disease.
06:22.000 --> 06:28.970
For instance, patients who have pancreatic
involvement may experience
06:28.970 --> 06:30.790
pancreatic exocrine or endocrine failure
patients who have.
06:31.690 --> 06:37.970
fibrosis aitis may experience renal failure or
enlarged aortic
06:37.970 --> 06:40.190
aneurysms at risk for dissection.
06:40.489 --> 06:46.880
Patients who have IgG4 related ophthalmic disease
may experience orbital swelling or pseudotumors
06:46.880 --> 06:53.489
that affection since IgG4 related disease can
manifest itself in so many ways the
06:53.489 --> 06:57.690
associated functional and structural damage
also varies.
06:58.320 --> 07:04.320
as a result of this potential for damage
patients with IgG4 related disease may experience
07:04.320 --> 07:09.790
frequent symptoms, distress and uncertainty
about their disease course.
07:10.609 --> 07:16.570
approximately nine out of 10 patients with IgG4
related disease will experience symptoms of
07:16.570 --> 07:19.149
distress related to their condition.
07:20.380 --> 07:26.220
If patients identify fear as a common experience
during the journey the burden of disease in
07:26.220 --> 07:32.459
associated distress is a combination effects
from the active disease as well as potential
07:32.459 --> 07:38.980
damage from the disease and its treatment as we
continue to understand IgG4 related disease the
07:38.980 --> 07:42.279
patients perspective is an important area of
focus.
07:43.059 --> 07:45.010
We have much more to learn in that regard.
07:46.029 --> 07:52.869
To summarize IgG4 related disease is a chronic systemic
disease that can occur in nearly any organ.
07:52.989 --> 07:59.350
It is progressive and characterized by
unpredictable flares that can damage new or
07:59.350 --> 08:00.790
existing disease sites.
08:01.220 --> 08:06.359
Thanks for listening please the three other
videos in this series from my colleagues for
08:06.359 --> 08:12.040
more information on IgG4 related disease.
including the pathophysiology of IgG4 related
08:12.040 --> 08:18.480
disease, the importance of a multidisciplinary
approach and goals of treatment and
08:18.480 --> 08:19.869
proactive Management.
