IgG4 related disease is a rare chronic fiber
inflammatory disease that is characterized by
unpredictable disease flares and can affect
nearly any organ in the body.
Im Dr Emma Culver, a gastroenterologist
hepatologist at the University of Oxford United
Kingdom. Where i will multidisciplinary team dedicated to patients with IgG4
related disease, including gastrointestinal
manifestations
such as Type autoimmune pancreatitis and IgG4 related
Colangitis Today Dr Calver and I are going to
walk through some of the rational for a
multidisciplinary approach to IgG4 related
disease and the importance of partnering across
specialties for patients living with IgG4 related
disease.
to see beyond the symptoms and think
systemically let's begin with a brief overview
of IgG4 related disease IgG4 related disease is
a multiorgan disease that can appear in nearly
any organ.
The organs affected 1988 related disease can vary
greatly across patients.
The most commonly affected organs include the
lacrimal and salivary glands,
the pancreas and the retroperitoneum, but many
others ranging from the orbits to the lungs and
the aorta can also be impacted.
The symptoms that patients present with depend
on the organ or organs involved.
However, IgG4 related disease can also progress
over time to affect new organs.
Dis Inflammation and fibrosis can lead to
structural and functional damage.
Awareness of how structural damage and organ
impairment manifests is critical important to
inform the timely diagnosis of IgG4 related
disease in your patients given the varied
clinical presentation and often multi organ
involvement several specialists might be
involved throughout the patient journey.
Rheumatologists and gastroenterologists are
often the primary clinicians diagnosing and
managing patients, although as we will discuss
shortly collaboration with radiologists and
pathologists is crucial to make a diagnosis as
well as other specialists based on organ
involvement diagnosis of IgG4 related disease may
be challenging.
symptoms often overlap with other conditions,
including autoimmune and inflammatory disorders
and malignancies.
There is no single fully reliable diagnostic
marker diagnosis requires careful assessment of
clinical, radiologic serologic and
histopathological features.
Let's walk through some of these features in
more detail to determine when IgG4 related disease
may be considered.
Generally speaking clinical and radiological
features of IgG4 related disease include
diffuse or localized swellings strictures or a
mass or nodule characteristic of IgG4 related
disease in my experience with gastrointestinal
manifestations a IgG4 related disease such as IgG4
related autoimmune pancreatitis We often see
diffuse pancreatic enlargement or focal masses
on imaging.
In many cases patients also have biliary
structures IgG4 related cholangitis.
These presentations can often mimic pancreatic
adenocarcinoma or cholangiocarcinoma and
therefore clinicians need to be cautious to
exclude malignancies through additional
assessments Emmanuel can you share typical
examples of characteristic imaging of IgG4 related
disease sure presentation we see on imaging is
retroperitoneal fibrosis.
As you can see here IgG4 related retropal
fibrosis can present on imaging as a soft
tissue announcement surrounding the aorta.
Assessing Sology and histopathology are also
crucial to distinguishing between IgG4 related
disease and its mimickers.
Thanks Emmanuel bring a great point lapworld to assess
serum I levels is an important component
of a diagnostic worker for IgG4 related disease
as only twenty percent to forty percent of
patients with IgG4 related disease present with a
normal baseline IGT level.
However elevations in serum IT levels can occur
in other conditions and therefore assessing
serology is just one of the many important
steps that are needed.
pathological features of IgG4 related disease
included dense lymphocytic and IgG4 positive
plasma cell infiltration and as you can see
here an increased ratio of IgG4 positive plasma
cells to IgG4 positive plasma cells.
Lastly typical tissue fibrosis such as a storm
fibrosis ohne Blistertip für weites can
often be observed given the overlapping
clinical features and the importance of ruling
out malignancies a multidisciplinary approach
to diagnosis and management can help clinicians
feel confident in an IgG4 rate.
Disease Diagnosis several organ specific
diagnostic criteria based on clinical
radiologic serologic and pathologic features of
IgG4 related disease have been developed
for instance gastroenterologists are likely
familiar with the His criteria for autoimmune
pancreatitis.
organ specific criteria can help diagnose a
single organ manifestation IgG4 related
disease is a multi-organ and progressive
disease.
Therefore it is critical to evaluate patients
holistically to understand the whole story of
the IgG4 related disease diagnosis.
Additionally, the AR Euler classification
Criteria for IgG4 related disease provide a three
step framework with high specificity and
sensitivity for classifying IgG4 related disease
of note These classification criteria were
developed for clinical trial use and while they
are a valuable tool in our toolbox says
clinicians they are not a diagnostic criteria.
however the criteria provide clinicians a
valuable framework when considering the
diagnosis of IgG4 related disease and may also
add in eliminating alternative diagnosis or
mimikers of IgG4 related disease.
The story of IgG4 related disease and patient
journey can be long and winding Patients might
have disease that progresses to involve
additional organs before they finally receive a
definitive diagnosis.
It's important to add that the patient journey
does not end once the patients are diagnosed.
This is a chronic disorder and most patients
will relapse after initial treatment.
Monitoring is often advised to ensure patients
do not repeat the cycle of referrals and
additional testing.
As a gastroenterologist it may be helpful to
partner with a rheumatologist or continue to
monitor the disease outside your organ of
specialty.
clinicians should consider an approach that
works best for them with the understanding that
disease relapse may occur and that flare
presentation can vary from initial diagnosis.
clinical vigilance and Partnership with the
multidisciplinary team can help to optimize
that patient's journey in summary today we
learned about the importance of a
multidisciplinary approach to IgG4 related
disease and touched on the following key points.
IgG4 related disease is a multi organ disease and
care coordination among specialists may help to
optimize diagnosis and management to reduce
risk of disease flare.
There is no single diagnostic test for IgG4
related disease clinicians have several tools
available to help them confidently establish an
IgG4 related disease diagnosis disease
awareness of disease characteristics and coordination specialists
help to improve patient experiences and
outcomes. Thank you for listening to my colleague
Emma Calver discuss related disease today we are
pleased to review this very important.
Please see other videos in this series from my
colleagues for more information on IgG4
related disease,
including Disease Overview Pathophysiology and
proactive Management of IgG4 related
disease.
