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Matthew J. Koster, M.D., summarizes the treatment, monitoring, and outcomes of Retroperitoneal Fibrosis.
Review when to consider IgG4 and ‘RPF’; Describe when to think presentations of IgG4 and ‘RPF’ are really something else; Discuss how to diagnosis and treat
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Internal Medicine Specialty
Rheumatology Subspecialty
Mayo Clinic
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Past Notes
Thank you for your attention and
staying for the last of the talks
today.
So in regards to the two conditions
that I'll be talking about so far,
related disease and retroperitoneal
fibrosis.
So I have no relevant financial
disclosures. But what I will say is
that there are no FDA approved
medications for either of these two
disease entities, and so any
medication mentioned will be
considered off label.
We'll be really going through three
specific objectives today.
One is to think about when to
consider either of these conditions.
And the second objective is when to
consider patients that are
presenting with futures suspicious
for this condition, but really have
something else and how to
differentiate that.
And then also to talk about the
difficulties in diagnosing these
conditions and a word on
treatments
starting with IGG for later disease.
So when we're thinking about the
concept of ECG for later disease, we
really need to think about the
characteristics of the disease
itself.
It's a chronic, insidious,
progressive disease, so it's not
something that's going to abruptly
occur.
So if you see something with a mass
or a tissue structure that's really
rapidly evolving, think
of other etiologies, particularly
infection or cancer
as opposed to age for related
disease, because that's something
that again is often more slowly
evolving over time.
And the other part is that it's
fibro inflammatory.
And that's part of the difficulty
both in the diagnosis of this
condition from histopathology
and also its response to therapy.
So some of the tissues that are
involved tend to have more of a
fibrous element, and the ones that
have higher fibrous elements
typically respond less to therapy.
And then the last characteristic
that's presumed autoimmune,
I say presumed because there's no
actual direct correlate to say
that there is an autoimmune process,
but we know that it's an immune
disturbance.
So we know that there is an increase
in ICG for both in staining and
also sometimes in serum.
But it's not necessarily the case
that there's actually an autoantibody
that's been developed.
There are disturbances that lead to
ICG for increase
and that's typically through class
switching. So T cells causing B
cells to be increased and then
increasing their volume of ICG for
production.
And then also an increase in the t
t cell response, which
is why some of these patients have
allergic findings or a topic
presentations and also the reason
why there's often high IGI
within their serum.
This slide I will leave up here for
a little bit while I talk about
issues because it's really important
to understand all of the different
organs that can be involved in this
condition.
And so it is a poly glandular
or multi systemic condition.
And I've highlighted the areas here,
the purple ones are the ones that
are most frequently involved.
So things in which over 50% of
patients will have to use the
blue are the occasionally involves
a to a 25% to 50%
and then the green being typically
less than 25%.
So the ones that you're really
thinking about with HGF related
disease are typically glandular.
So lacquer glands, prodded
glands of individual glands
and then the pancreas, the pancreas
is a large gland and exocrine gland
and then also the retroperitoneal.
I'll talk about what that looks
like, but that's typically Perry or
Titus. So in for renal abdominal
aorta, periodic thickening.
But several of these other areas can
also be involved and they can be
involved by mass lesions.
So things like in the orbit or the
lungs, but you can also have
inflammation within the liver or
biliary system causing features
suggestive of cholangitis
and then also the package meninges,
which can be difficult because
there's many different conditions
that can cause inflammation in that
territory.
One of the important things is that
only a quarter of patients may have
a single organ.
Often when it's a single organ, it
may be just the pancreas.
But most patients, more than three
quarters of patients, will have two
or more organs involved.
So if you identify one location,
you really need to go by clinical
history or examination and sometimes
searching through imaging to
identify if there's other organs
that are affected as that may
guide you on what treatment you may
decide to use.
So what are some patients who are
considered quite high risk for
HGF related disease?
So the ones that the GI doctors will
typically end up seeing these are
these patients with an idiopathic
or what they're called a type
one autoimmune pancreatitis.
So they don't have a reason.
They're not alcoholics, they don't
have calcium issues, they don't have
medication, precipitant.
And we'll see later on some images,
but often they'll have kind of this
diffusely boggy pancreas on imaging.
You can have patients again with
biliary inflammation or sclerosing
cholangitis, and that has to be
considered in the differential,
particularly if there's no
obstructive process.
As we mentioned, glandular
involvement. And often it's more
bilateral as opposed to unilateral
for having unilateral involvement.
I often think of things like
infection or malignancy, like
lymphoma
when we're thinking about serum for
and we'll talk about this as far as
the assessment, but it's typically
patients with high igg4 levels.
So when you're talking about serum
levels. Five times or
higher than the upper limit of
normal.
And each lab has
a different upper reference range.
Some are 86, some are 113.
Some are 121.
And so you really need to know your
upper reference range.
But we're really talking about
patients in the 400, 500
plus range as opposed to someone if
the upper limb. It's 86 and it's 91.
So in evaluating patients with ADHD
for the disease, it is important to
check the serum under G4, but you
can't rely solely on IHG
for level one is that can be
elevated from other etiologies, and
two is that it can be normal in
patients with this etiology.
When you're looking at patients in
general, about two thirds of
patients will have an elevated HD
four. And so it has a good
sensitivity, but a relatively poor
specificity.
Also, how you check it is important.
So there's different ways in which
you can assess for the ADG for level
one is looking specifically at
ECG for Subclass in and
of itself, and the other is looking
at immunoglobulin IgG
subclasses.
And so if you look at the
subclasses, which looks at one, two,
three and four, you can get a pro's
on effects of the time they're
getting to you, for
they may not have as much of an
increased amount that they're seeing
on the study and so can be falsely
lowered if you're looking at these
subclasses as opposed to be distinct
subclass.
The other labs that are helpful, as
I mentioned, is looking for
inflammatory markers and IgG
because there can be this t two
dominant response.
So elevated IgG values
increase the suspicion of this
condition and it can also be
complement fixing and so low
complement C for particularly can
be helpful in patients identifying
a higher likelihood of HGF related
disease.
Other labs that have listed in the
purple block on the right hand side
are largely looking for other organ
abnormalities. And so really we're
here looking for liver
abnormalities, kidney abnormalities
with a complete metabolic panel and
a urinalysis to look for concerns
for things like tubular interstitial
nephritis.
And then the other ones lists are
largely to look for.
The more common mimicry is that we
could see with some of these
presentations that for can mimic.
Among all of the different locations
of tissue that can be involved,
there is a common histopathology,
and this is what ultimately led
towards the identification of
multiple diseases that were
considered to be separate entities
being lumped underneath for
related diseases.
So looking at it here, we can see
that there is this kind of dense
lymph of plasma, acidic infiltrate.
Here was a vein and now
it's occluded with an inflammatory
infiltrates that's obliterate of
phlebitis.
And then this kind of basket weaving
of pink, this is what's called
storer form fibrosis.
So kind of this interwoven area
of fibrous tissue.
There was a consensus statement.
Now, this is about ten years old.
So 2012 that came up with what
are the histopathological features
that are suggestive or highly
suggestive of Igg4 related disease.
Now I would kind of label the slide
as clinical correlation advised,
because if you look at the
description, none of these say
conclusive for ADG for
the disease. And that's one of the
important things to take from this,
is that the diagnosis
of DG for related disease cannot be
hinged only on the biopsy.
A pathologist will use these
different cutoff levels
of different cells per high
power field that stain for igg4
based on not only the location
of the tissue, but also how
it was obtained.
So they allow for lower cutoffs
in FE in core biopsies
compared to surgical biopsies that
would have larger tissue.
So all of the areas highlighted in
green are ones in which
if they have in this circumstance
two or more histopathological
features listed here plus
specific cutoff levels, they will
label it as highly suggestive
or suggestive for DG for
the disease, but they will not say
it's G for the disease that's based
on the clinician taking all of the
data together.
But the ones that are kind of highlighted
in the tan, that's where they will
list probable for allergy
for the disease.
And you'll see this description in
the histopathology.
And often they will quote this
specific study for that.
Now, it's important to know that not
every tissue is going to have all
of these features.
So if you get areas that don't have
a lot of vascular ity, you're not
going to see veins and therefore you
may not see the ability to
phlebitis.
So typically you will end up seeing
at least in dense lymph,
a plasma acidic infiltrate.
But you may not always end up seeing
enough of the story from fibrosis,
depending on the tissue.
And if there's no veins and you
won't end up seeing features of
ability to phlebitis
when talking about cutoffs, specific
cutoffs can be very confusing.
Try to remember this after
walking out of this lecture.
After I move from the slide, you
have to reference it because of all
the different characteristics and
specifics.
So if I were to leave you with one
thing to consider, it's more of a
ratio. So if you have a higher than
40% ratio of IGG
for staining cells to IgG staining
cells, that has a higher suspicion
and greater specificity for things
that would be concerning for you,
for the disease.
So again, just to reiterate, if you
have do for staining either by high
power field or by ratio,
that in and of itself does not
guarantee ADG for the disease.
You really need to think of other
things that can lead deposition of
Igg4 and often we can see this just
in chronic inflammatory states.
So there are inflammatory conditions
in which I will say ANCA vasculitis
is one of the more common we see
some IgG for staining in the sinuses
that can be nonspecific and in
those patients if they're positive
manka serology specifically you
may require additional biopsies or
looking to other areas to assess
for that entity.
In addition, there are other
lymphoid proliferative disorders
that can have low level staining,
and you may require having to go
back to the pathologist and ask them
have they done appropriate stains to
exclude these if it was not listed
for them to look into these things
at the time of their initial
evaluation?
So again, very complex
to end up diagnosing these patients.
But you have to understand that it
takes the clinical elements, the
serologic elements, although there's
some limitations with that, the
pathologic elements, even though
they can't conclude on the biopsy
and then radiographic features.
And so understanding the
commonalities of this condition is
important and looking at those and
bring it all together.
There is a classification system
for DG for related disease.
This is mainly for research and
inclusion and studies.
It's not a diagnostic classification
criteria.
And I will kind of just summarize
on this slide and then we'll show
the nitty gritty details
in the subsequent slides that are
quite complex and confusing.
So essentially start with classical,
right? So the areas of the
territories that we mentioned in the
beginning, slides of what organs can
be involved that have a typical
them for plasma infiltrate either
on biopsy or radiographic features
suspicious.
But then this is one of the few
classification criteria in ACR
where their next step is excluding
mimics. So you have to go through
all the possibilities and try to get
rid of that before you
even move on to the next, which
would be a scoring system for
inclusion, which is quite difficult
to do.
And then you have to add those up
to 20 or more to end up having
the possibility of the person having
ADG for from a research
classification standpoint.
So again, this is some of the
additional details.
We're not going to go into all of
these specifics. They have these in
the handouts for you to look at
additionally.
But some of the important things to
consider are if they're presenting
with fever or profound weight
loss or significant constitutional
symptoms, those are often not
typical of G4 related disease.
And if they've been started on
steroids therapy and had no
response, that's also a warning
flag.
In addition, they suggest checking
all of these labs and
antibodies and excluding ones
that have positivity or concern for
other etiologies.
And then from pathology, again,
really trying to avoid
having some overlap or mimics from
ANCA associated vasculitis
again with the necrotizing
vasculitis or predominant
neutrophils inflammation
and also history, acidic
infiltration, looking for things
like time Chester disease, which
are often commonly confused
but can have some nonspecific
staining.
And then you go into this complex
scoring algorithm.
We won't go each individual box, but
you can see how there's very defined
cutoffs and criteria as far
as what then gets what points,
and then adding up all these points
greater than 20 would end up
including you as classification
purpose of G for that disease.
So this highlights the complexity
even within people who are dealing
with this in a routine basis.
In the development of the
classification criteria.
One of the important things that was
found is that they use a United
States and European cohort and also
a Japanese cohort.
And looking at those two populations
together, they identified four
separate clinical phenotypes and
we'll walk through that. So one and
the most common is pancreatic
hepatobiliary.
So this is about just roughly less
than one third of all patients with
agency for their disease.
So maybe difficult to see
from the back, but this is kind of
a dilated, boggy pancreas.
So this is that autoimmune
pancreatitis.
These typically are men
are often more white than
Asian and they're typically older,
40, 50, 60.
They will have some typical
elevation of age for the disease
compared to normal values and often
steroid responsive and other
treatment response as well.
In comparison to about a quarter of
patients. This is kind of a
characteristic Perry
or Titus, so anterolateral.
And for renal abdominal aorta, this
person has urethral stents because
of obstructive disease.
These are also male,
white, forties to sixties,
but they're for maybe minimally
elevated or completely normal.
And these often don't respond as
dramatically.
They may have some reduction of
tissue, but not full resolution.
And that's because often it's more
of a fibrous collection of tissue,
less inflammatory.
And then about a quarter of patients
have what's called head and neck
limited. These patients tend to be
women that were seen more in the
Asian group, in the
validation cohort, and
they will typically have some
elevation of age for the disease.
But if they have thyroid
involvement, particularly with
Ribose Thyroiditis, then
that's more of a fibrotic process
and may not have full resolution.
And then there's this fourth
category, about 20% of patients,
it's Nicholas syndrome.
Some Nikolic syndrome is bilateral
lakmal carotid and sub mandibular
gland involvement.
And then they also have some
systemic features.
Now the systemic features tend to be
things like pancreas, lung,
etc.
Again, this is also male and older,
but they have the highest of all of
the different subtypes for IGT four
levels.
And they also tend to be treatment
responsive.
Now, just because of sheer time, we
don't have time to go through all of
the different treatment options and
the nuances and the limitations of
the studies given most of them are
retrospective, few prospective
studies.
But there was a consensus statement
that was put out in 2015.
There hasn't been a whole lot of
well-designed studies since then,
but essentially the first line
continues to remain modest a high
dose glucocorticoids 3 to 4 weeks
and then tapering that down over 3
to 6 months depending on response.
If they can't tolerate or can't have
vertex or prednisone, then you could
consider rituximab for induction
therapy.
Maintenance regimens have even less
data.
The Japanese groups typically end up
doing some low dose glucocorticoids
5 to 7 a half mg, often long
term.
But there is a push, particularly in
the United States and European
countries, to get patients off of
glucocorticoids.
And so a use of kind of a modest
disease modifying agent can be done,
but there's very little data as
opposed to which one's better than
the other and whether or not they're
truly effective, except in a few
populations that were mentioned.
The point is to try to limit
aggressive immunosuppression.
So that's why for maintenance
therapy, it's often suggested that
rituximab is considered as a backup
option as opposed to a routine
option.
So here are some just general
guidelines. So in patients who are
asymptomatic with glandular
issues, you can watch in these
patients, patients who do
have quite high DG for levels
or IGF levels or if
they're kind of multi-organ
involvement. Those patients have
been observed more in retrospective
studies that if you use upfront or
early disease modifying agents,
you have a better chance of
remission off of prednisone
again if they can't take prednisone
or if there is contraindications to
using prednisone or if they failed
with prednisone.
Rituximab has been shown in open
label prospective studies to be
helpful and if they're not
responding to any of this, really
reconsider whether you're truly
certain that they have you for the
disease or you need to look into
some other options.
There are ongoing therapies
predominantly looking at B-cell
depletion therapies, but there's
also the slam F seven inhibitor
to the amounts typically used in
cancer treatments.
And that is being done now in a
phase one phase two study
that's led out of Harvard or John
Stone that we're participating in.
Here's a representative case of a 20
year old female who was sent to me.
She initially just came with some
bilateral, acromegaly and
enlargement and really was largely
asymptomatic.
She did have a little bit of parotid
and some interviewer gland
thickening, but really didn't even
mention it when you asked her
relapse were notable for high IgG,
high for low complement,
quite high IGI
and her ESR was elevated but
CRP was normal.
Now she had been seen for this for a
couple of times by different
providers, went to allergy because
of the high IGY and they just said,
well, we don't really know and just
keep watching.
So when you ask her, she really
doesn't feel unwell.
So would you end up treating her or
just watching her?
The point is, you really need to
understand what else is going
on in these patients. So if you do
imaging, she actually had quite
significant even though
asymptomatic, growing glass
opacities and bronchial wall
thickening and also a few pulmonary
masses.
In addition, she had left greater
than right Inguinal and nothing.
So because of the concern of
potential for lymphoma
or other process, we did biopsy both
locations, lung and inguinal
lymph nodes and both of them.
So very high levels of ADG
for both on high power field and on
percentage staining.
So again, her initial
after four weeks of 40 milligrams of
prednisone near resolution of the
pulmonary findings and we put her on
a disease modifying agent again
because of issues with the high
four levels, multiple organ
involvement as she tapered down,
she did have recurrence of symptoms
as she went off of steroids.
And so then we did do rituximab and
she did have significant
improvement.
Because this can affect many
different organ systems.
You have to kind of guide the
patient as far as what to look for
because not all patients will only
stay in the single organs that they
have and they may manifest in other
areas, particularly if they're not
on treatment.
I do recommend watching these
patients every 3 to 6 months
clinically. If they're doing well,
you can move it out closer to the
six months and then labs that
are helpful. If they had elevated
IGT four or elevated IGT
or low C4, those are helpful to
monitor. But if they've always been
normal, the repetition of them may
not be helpful if they're overall
asymptomatic.
And imaging may be required in some
patients. If you just can't get
an assessment of whether or not
there's some abnormality based on
clinical exam, physical
or in patients with labs.
So I'll take the remainder of the
time to look at retroperitoneal
fibrosis.
So when we're talking about
retroperitoneal fibrosis, it's one
of those difficult terms or I think
it means different things to
different people.
When I think of retroperitoneal
fibrosis, I'm thinking more of
chronic peri irritated, so kind of
thickening around and for renal
abdominal aorta kind of
in this perspective.
Now, not all patients have this
amount of avidity, but we're talking
about patients, again, often more
peri aortic disease.
When a radiologist talks about
retroperitoneal fibrosis, they're
just talking about any soft tissue
thickening that's present anywhere
in the retroperitoneal.
And there are many different
ideologies that could be there,
idiopathic causes or the
bulk of patients with this.
And interestingly, the nomenclature
still includes HD four
as a cause of idiopathic,
although if it's ADG for later
disease causing it, you would think
that that then is a separate entity.
But the nomenclature is not cut up
yet.
It's a rare condition.
So about 1 to 3 per 100,000.
We've seen that in a couple of
European cohorts and then we've
recently replicated that similar
data through the Olmsted County
cohort in Rochester.
There is a male predominance
compared to females and often
this is identified between the fifth
and seventh decade.
There are several secondary causes,
as I mentioned, some of the drugs
that can be used or have been
associated with this we don't use
commonly anymore.
But important to understand
infections are supposed are also
important to know is that you can
exclude them often by
stains or by serology
and then understanding again that
there are other things, including
malignancies, that you really need
to know and push for on biopsy
or assessment of other organs
really to identify this, because if
they're not identified, then
treatment with steroids or
immunosuppressive therapy will not
help these patients.
They need often targeted therapy to
assist.
So we have just recently expanded
actually Dr. Kermani is initial
cohort. So she had looked at this
from 1996 to 2006
and we've now moved it from 2006
to 2019.
This will be when printed, the
largest cohorts
in North America of patients with
Perry or Titus.
But most of the patients are
symptomatic.
But there is a subset about 10 to
15% that end up just having it found
on radiology.
The problem is that the symptoms are
quite nonspecific.
So most that have belly pain, back
pain, flank pain fatigue, some
lower extremity edema, these are
often things that are dismissed by
primary care and not further
evaluated. So they may have these
things for a while until they then
present with significant pain,
adrenal insufficiency because of
obstructive or apathy.
Again, we demonstrate similar
characteristics about patient
profiles and also similar
to what has been seen as far as
concern of as best as exposure.
And what I would say is that the
more recent patients have not had
asbestos exposure largely because
of its eradication and their age.
And then there does seem to be a
correlate with tobacco, a large
portion of patients and having a
history or current use of smoking.
Now when we're talking about where
things are looking at, so this is a
kind of just a representation,
again, anterolateral and the
inferring of not only order but
understanding when that thickening
occurs, it can either pull in the
ureters or it can obstruct them
as they're going over the pelvic
brim and then going down into the
bladder.
And ultimately, what that can occur
in a large majority of patients is
that they can develop obstructive
neuropathy and renal insufficiency.
And depending on how soon this is
identified and taken care of, can
end up leading to long standing
kidney damage.
So that's why I will go through the
the steps that I would suggest in
evaluating patients with
retroperitoneal fibrosis.
The first is that when this is
identified, the most immediate step
is assessing for renal insufficiency
and kidney be compromised.
And if it's present, it needs to be
dealt with urgently.
So if they have hydrogen of process
and renal insufficiency that needs
to be decompressed either through
urology with a reader extension
on the right or in sometimes
in the frost me tubes, as done often
in our institution by interventional
radiology to allow for the urine
to flow and maintain the renal
function.
And then you can later on deal with
the other ramifications of the
disease.
The second step, even though it's
not very common, can be quite
significant, is venous involvement.
So if these patients have either
unilateral or bilateral lower
extremity edema.
The concern is that if you do lower
extremity Doppler studies, you're
not going to see a clot in all these
patients because it's more proximal.
Here shown picture
graphically a left common iliac
artery occlusion. And here is one of
the patients shown with
interventional radiology angiogram
here showing the kind of proximal
portion and the distal portion
in which she had extrinsic
compression from that
radiating mass from the iliac
artery, compressing that area,
ultimately required thrombolysis
anticoagulation.
And this person had bilateral common
iliac vein stents and did
very well. And often these patients
do once that's accomplished.
The third is assessing extent.
Now, often if folks have belly pain,
flank pain, you'll get a belly
scan and then you'll see some
thickening. But what about the rest
of the arterial tree?
So again, in some patients, if they
have ICG for their disease, that
will increase the likelihood of
them having kind of higher up
involvement, thoracic aorta or the
peri coronaries.
And so this was a patient who was
actually followed by my predecessor
for years because they had
abdominal abnormalities.
But then as soon as I saw him for
the first time, we extended and they
actually ended up having a near
subtotal occlusion of the right
coronary artery that then went to
urgent stenting.
In addition, you can see thoracic
aortic involvement, often kind
of more of this CRECENTE involvement
as opposed to fully circumferential.
And then once all those are
dealt with, I think then you can
really launch into what's going on
and what's going to be the diagnosis
for these patients and how you're
ultimately going to manage them.
While you'll read some
recommendations, that biopsy is not
necessary. If there's classical
findings of retroperitoneal fibrosis
or PERI irritates, I will say how
many of us know what classical
findings of Perry or Titus are
unless you see a lot of these.
So therefore, I would say if it's
possible, biopsy the tissue
or if there's some added apathy or
atypical features, biopsy those
tissues as well, because it's
important to differentiate other
etiologies,
and particularly when you're
thinking about other conditions and
things that may push you to consider
different causes.
The main things that I would go
through is that if the location is
atypical.
So again, for more Perry or Titus
Anterolateral and Farina
abdominal aorta, but if it's outside
of that, if it's in the soft tissue
in the retroperitoneal and the
pelvic sidewall, the sacral areas,
peri duodenum, these are all
atypical lesions and they
may be associated with other
pathologies, particularly
malignancy.
In addition, often things that are
extending from the aorta,
they may extend
and they may pull things in, or they
may extensively compress, but they
don't infiltrate.
So if you have a radiologist who's
describing things of infiltrate of
into the soft tissue or into
the muscle spaces, that's
concerning, particularly for
infection or malignancy.
In addition, if they have a lot of
constitutional symptoms or bone
pain, consider that as well.
I typically get a detailed past
medical history looking specifically
about cancers, about whether they've
had them, because if they've had
them before, think of that as a
possibility and really push
for excluding it.
Here are the labs that I would
suggest, which we've talked about in
previous slides.
So I've had several cases sent to me
in which patients were labeled
locally as having retroperitoneal
fibrosis and ultimately had
something different.
This is a gentleman who initially
had bilateral obstructive
neuropathy, who had stents placed.
He actually was diagnosed with
lymphoma when he was having severe
back pain and ended up having back
surgery. And during the back
surgery, which is what this horror
is, they did a biopsy and found
diffuse large B-cell lymphoma in his
spine.
And I went treatments and then the
local oncologist was saying that he
was in remission from his lymphoma,
but he developed this obstructive
your apathy. He has RPF, he
needs steroids. Refer to Mayo
now. The concern is that you can see
the distribution, that it is
layering over the vertebrae
and it's infiltrating into the
spinal muscles.
So again, of diseases,
posterior or pre sacral
or peri lumbar.
Those are concerning.
So it was very challenging to
diagnose this patient because we
needed general surgery and also
musculoskeletal oncology surgery
to go in and get adequate samples.
But he ultimately was diagnosed with
an infection that was emanating from
his hardware and that
required antimicrobials,
not prednisone
or immunosuppressants.
Here's a different gentleman who
came up from Texas, slapped
down a manuscript about allergy
for the disease and told me that's
what he had.
I asked if he ever had a biopsy.
He said no.
He had some more significant and
apathy in his lower pelvis.
This is kind of at the iliac
bifurcation.
He has a little bit of a vanity.
And so we biopsied him and
he had marginal zone lymphoma.
And then finally on this slide, this
is a different patient.
Again, obstructive your apathy.
You can see with no frost me tubes.
This person has no significant
period aortic thickening
but had this significant soft tissue
mass that was by the duodenal
area and the retroperitoneal.
So again was on high dose steroids 3
to 6 months.
No significant improvement being
considered for secondary or
refractory
chronic periodontitis, a
retroperitoneal fibrosis.
We were able to end up getting an
endoscopic ultrasound through
EGD and biopsies that confirmed
evidence of metastatic
breast cancer and she had previously
had been diagnosed and treated
effectively over ten years
previously.
The last minute that I'll describe
to you here really graphically,
so this is at the level of superior
mesenteric artery.
There's significant thickening
around the entire portion of the
aorta, but more importantly,
there's significant thickening in
the PERI network space.
So when you look at it, there's this
long sheath of thickening
circumferential around the aorta.
And so that was described
and is called the coated aorta.
And then these projections into the
kidney, peri networks faces
was called this hairy kidney.
This is not typical of
retroperitoneal fibrosis.
And if you see this, you really need
to think about this upcoming
condition that I'll describe.
So this patient, we ended up
proceeding even though he was
asymptomatic, to get a bone scan and
can see significant uptake of the
technician in the distal femur and
the proximal tibia.
And then we biopsy that peri network
area and also the distal femur.
I saw these foamy macrophages,
this history of sites,
and this is Earth Time, Chester
disease.
This used to be thought to be an
inflammatory condition, but really.
A malignancy, an AIDS targeted
therapy. He was BRAF v 680
positive and underwent direct IT
therapy and did well.
So how do I typically approach
patients with quote unquote
idiopathic RPF?
Typically from this, if I've
excluded the other mimics, if there
aren't things that we need to lean
into as far as other etiologies, I
will start them on high dose
prednisone and reassess them in
about eight weeks radiographic
to assess for response.
And by response it's typically a
reduction in soft tissue thickening
20% or more.
If that is present,
then we continue with
therapy, often initiating a disease
modifying agent to assist in
successful tapering of steroids.
And I will bring them back at
different cut points, 2010 and off,
and then once they're off every 3 to
4 months for about a year and then
approximately every six months for a
couple of years after that, ideally
having them stable off of
prednisone on a disease modifying
agent if they've been stable
for about a year and a half to two
years off of steroids, then
I'll talk about withdrawing the
additional therapies,
patients that may not respond.
So if you get a PET scan, if
you're able to ahead of time
and if you get a biopsy, you can
correlate. If they have a lot of
fibrosis on biopsy and low FTD
avidity, those are ones that may not
have as dramatic of a response.
And so that's something to be
anticipated.
But if they have no response at that
eight weeks, then really think to
yourself whether you feel
comfortable, whether this really is
idiopathic RPF or chronic
periodontitis, or there's something
else going on
for our urologists
at Mayo.
They don't like to go right away
into ureter or ELISA surgery, moving
the ureters away from the fibrotic
mass. They like to give us an option
for at least try to have us do
medical therapy for close to
a year to see that we've maximized
therapy.
And they don't like to go into
inflamed state because if they do,
the question or concern that they
have is that they may provoke a
surgical complication and failure
of the surgery.
And so they prefer to have us
optimize those patients first.
So some just overall outcomes to
kind of guide patients.
These have been seen within
our large cohort that I mentioned.
So about 60% of patients will have
a reduction, but not resolution
of the soft tissue thickening around
the aorta.
A few of them will have absolutely
no change or increase
when you're tapering them off of
glucocorticoid therapy or
withdrawing them from a disease
modifying agent.
It's possible, but uncommon for
worsening of the soft tissue
thickening in patients
that do require stents or
nephrectomy tubes.
The majority, two thirds, will be
able to have them successfully
removed, either through medical
therapy as opposed to surgical
therapy. But if surgical therapy is
required, we do have quite
a high success rate in trying to do
patients who have been on therapy
first and then go
to surgical outcomes.
Often folks will ask, what about the
aorta? Will it become aneurysm?
Typically no. If you think about
this, this fibrous tissue is still
present. That kind of buffer is the
strain of the stress of the aorta.
So in our group, only 3%
of patients that did not have an
aneurysm at baseline proceeded
to develop an aneurysm over time.
And again, we're watching these
people for several years with
radiographic imaging.
So these are my parting words.
I tell this to our fellows as well,
that IGT for the disease and
chronic period is very complex.
The most important thing is to
really exclude other possibilities
because those will require different
therapies and patients will have
better outcomes.
If we identify those early
and if it's not responding well,
never feel like you can't go
back and reevaluate re biopsy,
re image, re expand your field
of focus or get other people
involved.
And so at that I'll end up ending
I think we're a few minutes over,
but hopefully that answered some
questions that people would have had
during this Q&A time.
Thank you for your attention.
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