I'm also the founder and executive.
IgG4 Forward Foundation, the first organisation
dedicated specifically
to IgG4 related disease advocacy research and
support today I'm going to discuss what IgG4
related disease is, including its clinical
presentation and typical disease course I will
also elaborate on how our understanding of IgG4
related. As a systemic
condition recently emerged.
IgG4 related disease is not a new disease.
Its first recognition in the medical literature
appears to date back to 1892 the description of a
forty two year old man a farmer who had
painless bilateral swelling of lacrymal glands
and his major salary glands. However
understanding of it as a systemic disease did
not occur until 2003. When we
researchers from Japan described autoimmune
pancreatitis as part of a larger systemic
disease that we now know as IgG4 related disease
over the past two decades our understanding
of IgG4 related disease has evolved
significantly in 2011.
IgG4 related disease was recognized at the First
International Symposium on IgG4 related Disease
in 2019 Classification Criteria for IgG4 related
disease were established.
now let's move on to what we know today in terms of
the epidemiology of IgG4 related disease.
IgG4 related disease can affect both men and women
tends to have a male predominance.
The typical age diagnosis is fifty to seventy
years but IgG4 related disease can appear
in individuals of essentially any estimating
the total impact of IgG4 related disease is
challenging since the epidemiologic data are
limited.
Recent data from the US show that IgG4 related
disease has an incidence rate of approximately
one per 100 thousand individuals and a
prevalence of approximately five per 100
thousand individuals IgG4 related diseases.
therefore has a similar incidence and
prevalence as other rheumatic diseases such as
associated vasculitis and systemic sclerosis.
Fortunately we are continuing to learn more
each day through research and growing awareness
in the medical community.
so what exactly is IgG4 related disease IgG4
related disease is a chronic systemic disorder
that is characterized by tumor inflammatory
masses and tissue fibrosis IgG4 related disease
can present in
nearly any organ and frequently affects
multiple organs simultaneously by the time it's
diagnosed at the time of diagnosis.
patients an average of three affected organs
and over the course of the disease this may
progress to include additional organs.
Hallmarks of the disease include an elevated
serum IgG4 concentration,
increased numbers of IgG4 positive plasma cells in
tissue and circulating plasma blasts.
Most patients have elevated serum IgG4 levels at
baseline.
20 to forty percent have normal values and this
does not rule out the possibility that IgG4
related disease might be present.
The diagnosis of IgG4 related disease is
challenging and may lead to a prolonged patient
journey with misdiagnoses of cancer not
uncommon.
The clinical presentation of IgG4 related disease
is heterogeneous and it can progress undetected.
It can occur in nearly any organ, but some organs are
more frequently affected such as the pancreas
bile ducts, retroperitoneum, lacrymal glands
and major salivary glands.
The pancreas is one of the most frequently
impacted organs.
It is affected in approximately forty five
percent of patients and is often accompanied by
biliary strictures patients with IgG4 related
disease also occasionally.
disease affecting the orbits lungs or kidneys
as you see on the image other organs can also
be involved but may be less common overall it
is important to know that IgG4 related disease can
occur virtually anywhere in the body which make
ongoing management and monitoring challenging a
focus on addressing the immediate symptoms of IgG4
related disease as opposed to chronic
management could
contribute to unpredictable flares and disease
progression because of the insidious slow
moving nature of IgG4 related disease
approximately sixty percent of patients have
irreversible organ damage by the time they are
diagnosed awareness of how organ damage
manifests itself is critically important.
to informing the timely diagnosis of IgG4 related
disease Disease recurrences often cause damage
in new sites of disease or in organs previously
affected by the condition.
Patients may have a higher chance of relapse if
they have risk factors such as multi organ
involvement or elevated IgG4 IGE or blood eosinophil
counts at
diagnosis.
in some instances relapses may occur when
tapering or after withdrawing glucocorticoid
therapy.
with the unpredictable nature of IgG4 related
disease it's difficult to determine and when it
will advance to irreversible disease.
Patients can experience a variety of
manifestations related to the functional and
structural damage associated with IgG4 related
disease.
For instance, patients who have pancreatic
involvement may experience
pancreatic exocrine or endocrine failure
patients who have.
fibrosis aitis may experience renal failure or
enlarged aortic
aneurysms at risk for dissection.
Patients who have IgG4 related ophthalmic disease
may experience orbital swelling or pseudotumors
that affection since IgG4 related disease can
manifest itself in so many ways the
associated functional and structural damage
also varies.
as a result of this potential for damage
patients with IgG4 related disease may experience
frequent symptoms, distress and uncertainty
about their disease course.
approximately nine out of 10 patients with IgG4
related disease will experience symptoms of
distress related to their condition.
If patients identify fear as a common experience
during the journey the burden of disease in
associated distress is a combination effects
from the active disease as well as potential
damage from the disease and its treatment as we
continue to understand IgG4 related disease the
patients perspective is an important area of
focus.
We have much more to learn in that regard.
To summarize IgG4 related disease is a chronic systemic
disease that can occur in nearly any organ.
It is progressive and characterized by
unpredictable flares that can damage new or
existing disease sites.
Thanks for listening please the three other
videos in this series from my colleagues for
more information on IgG4 related disease.
including the pathophysiology of IgG4 related
disease, the importance of a multidisciplinary
approach and goals of treatment and
proactive Management.
