Next, we’re moving on to
small vessel vasculitides.
Under small vessel, the way that we
way will divide this is ANCA
and non-ANCA association.
ANCA stands for antineutrophil
as we shall see, and there it is.
Please make sure you know what
ANCA stands for—antineutrophil,
or neutrophil, cytoplasmic antibody.
There are 2 major types of ANCA’s
that we shall take a look at.
You probably have gotten accustomed
to seeing both p- and c-ANCA.
You will no longer call it that.
You will know that p in ANCA
stands for? Perinuclear.
And as we shall see, this p-ANCA,
and now called MPO-ANCA,
in other words myeloperoxidase;
whereas if it’s c, c-ANCA,
that c stood for cytoplasmic
we, however, are now calling it with the
third letter of the alphabet, A-B-C, yeah. C.
So PR3-ANCA, proteinase 3,
as we shall see.
The manifestations, small vessel,
and let’s say that it’s cutaneous
blood vessels, skin manifestations.
Muscles and nerves could be affected.
Lungs, big time, and therefore, we know
what we used to call Wegener,
which now we will call, technically,
granulomatosis with polyangiitis.
You’ve heard of hemoptysis—lungs,
you also have hematuria—damage
down to the glomerulus.
Here, we’ll take a look at ANCA-associated
vasculitides, and here they are.
There are 2 forms—I’m going to walk
you through this step-by-step
so that, once and for all, this is
firmly planted in your head.
First, the p in p-ANCA
stands for perinuclear.
The c in c-ANCA is cytoplasmic.
Now we have to get more detailed.
The p in p-ANCA is MPO positive
Lots of p’s there to help you out.
I’m going to give you an
example of what’s p-ANCA positive
or PR3-ANCA versus your MPO-ANCA.
MPO-ANCA would be something like,
you’ve heard of eosinophilic
We’ll talk about Churg-Strauss,
or microscopic polyangiitis (MPA).
Don’t worry, that’s to come.
Whereas, if it was c-ANCA,
I’d like to remember this as being the
third letter of the alphabet, or PR3.
PR stands for proteinase.
An example for this, of c-ANCA or PR3-ANCA,
I need you to get in the habit
of knowing that it was formerly
We now call it GPA or granulomatosis
Now these are the antibodies that
are thought to be pathogens
in these vasculitides of the small vessel.
We often times call these ANCA-associated
Perhaps, you’ve asked yourself
what does that even mean.
Pauci means little, okay, by definition.
So could there be immune involvement
in these ANCA-associated
Absolutely, but is a significant no.
So it’s either non-immune or, technically,
little immunity involvement
meaning Pauci immune.
Whereas, let me give you an example
or a pathology for comparison.
is a pure immune complex
small vessel disease.
But let’s first complete our discussion
of ANCA-associated vasculitides.
Our first one is granulomatosis
I’ve mentioned this a few times now.
Formerly called Wegener.
Now whose the patient walking through
the door? A young patient? Sure.
And you take a look at their
nose and it looks like
something that you want to get on a horsy.
What do I mean? Saddle nose. Literally,
there’s going to be necrotizing destruction.
You see my ugly nose with the septum here?
Well the nasal septum could then
be destroyed and necrotized.
And when it does, it takes
on a saddle type.
In other words, a depression
as I shall show you.
Now, you want to divide
the issues in the lung
for GPA into 2 parts.
These are the 2 parts that you must know.
I want you to first predict, and then I’m
going to show you, so pay attention.
We have, #1: Recurrent sinusitis,
so that will be the upper parts of
the airways. Recurrent sinusitis.
#2: In the lower parts of the
lung that could be affected,
the patient is now presenting
Please know those 2.
Thirdly, small vessels down in the
kidney may also be affected
and now the glomerulus is undergoing
necrotizing type of destruction.
So when the glomerulus gets
guess what’s passing through there
easily? Blood. Hematuria.
So we’ve got hematuria,
hemoptysis, and sinusitis.
Let’s take a look at
We have recurrent sinusitis #1,
#2 I told you about hemoptysis,
Those are the 3 that you’re taking out
of the clinical manifestation.
In addition, you’ll have new ear tubes,
elderly patients meaning
Look for that as a possible clue
either when the patient is
walking through the door
or you’re reading about this patient.
In addition, I told you about the nasal septum
and might undergo necrotizing destruction,
we call this saddle nose.
What does a saddle mean?
Saddle of a horse; depression.
And skin issues, oftentimes, purpura.
Diagnostic study: Remember it used
to be called c-ANCA
but now we call it PR3-ANCA,
and what is PR3?
Or what does PR stand for? Proteinase.
On biopsy, what are you going to find?
Look at the name. Granulomatosis.
Not exactly sure?
There are a couple of theories out there as
to what’s causing granuloma, but you do.
You have to find granulomas and there
will be pulmonary hemorrhage,
and Pauci-immune. There you have it.
What’s Pauci mean? Little.
And GN stands for? Glomerulonephritis,
giving you the hematuria.
Those are some of the diagnostic tools that
you must find, most importantly the PR3.
Management: Well, you use
cyclophosphamide to induce
yes, cyclophosphamide to induce
and methotrexate to maintain.
In addition, you have steroids
and got plasmapheresis,
and ultimately—be careful though,
for organs that are threatened
by the disease itself.
Our second major ANCA-associated issue here
is clinically indistinguishable from
granulomatosis with polyangiitis.
There are a couple of things here
that I wish to point out to you
to make your life a little bit easier.
The granulomas might not be as prevalent.
The granulomas that you find
in granulomatosis with polyangiitis,
and if you don’t mind,
I’m going to start calling it GPA.
But the granulomas that you find in GPA
are not as prevalent in MPA.
You’re going to take this MPA when we
get into diagnostic column here.
It will be positive for MPO-ANCA.
What ANCA was positive in GPA? PR3-ANCA.
So right off the bat, I’m giving
you some clear distinctions
of how you can differentiate
between GPA and MPA.
Identical clinical manifestation;
however, the granulomas are not going to be
as pronounced in MPA, if at all actually.
MPA, microscopic polyangiitis, is MPO-ANCA.
It used to be called p-ANCA
And biopsy most likely will not
show granuloma. Is that clear?
I couldn’t emphasize that enough.
Management here, pretty much on the
same token as your GPA
including your plasmapheresis, and once
Continued discussion of ANCA-associated
small vessel vasculitides.
We have our third most common
or the one that often appears,
would be eosinophilic granulomatosis
Eosinophilic is what you’re
going to focus on.
None of the other, the other ANCA-associated
pathologies which, what were they again?
GPA and MPA.
Isn’t it nice to actually have something that
you can abbreviate and know what it means?
So we have GPA and your MPA.
In both of those clinical manifestations
and diagnostic tools,
we did not—did not, point out
So as soon as you start seeing eosinophils,
and you’re thinking about
issues with ANCA’s,
then eosinophilic granulomatosis, used to be
called Churg-Strauss, should come to mind.
So now as soon as you
think about eosinophils,
and how is the patient going
to present most commonly?
Bronchospasm type of issues. Correct?
So elderly patients with new onset asthma.
So look for bronchospasms. Allergies.
That should be clear now that
is completely different from its clinical
manifestation of both MPA and GPA.
Diagnostic tools: We have eosinophilia.
A chest x-ray might be abnormal. 50% of your
patients will then be MPO-ANCA positive.
So then you have 2 conditions which we have
MPO being positive, what are they again?
MPA, microscopic polyangiitis, and
On surgery, lung biopsy is Gold standard
and here you would find your
Look for that, look for that,
look for that.
Management here, steroids would
be the most common,
cyclophosphamide to protect those organs
that are threatened by the disease.
What you’re looking at here is a manifestation
called leukocytoclastic vasculitis.
It might be seen with eosinophilic
The picture on the left—we’re getting
closer to our description
of other vasculitides.
The picture on the left is showing
you purpuric rash.
Now what purpura means to you is the fact
that there is vasculitis taking place
in which there is a leakage of blood
from the blood vessel and;
therefore, appearing as a rash. Purpuric.
Not petechiae but purpuric. Purpura is
a little bit bigger, isn’t it?
Later on, we’ll talk about purpura
when we officially enter
and we’ve left ANCA-associated and go
into our non-ANCA-associated,
and well this is a patient’s leg
who’s a little bit older.
If later on I give you patient’s leg or
you’re given a child’s leg with purpura,
then you should be thinking
about anasarca purpura.
But here, this is GPA.
Remember, you can have little blood
vessels and you can have purpura
Absolutely. Be careful.
So you want to observe the background
of who this patient is;
where do you think this patient is coming
from, age so on and so forth,
and you can tell a lot.
The patient on the right, the arrow is
pointing to a depression of the nasal septum.
What happened in granulomatosis
That’s your saddle nose. There is
necrotizing destruction of the nasal septum
and a depression that I was talking
to you about earlier.
Also manifestation of granulomatosis
but could you also find it with MPA?
Yes you can. Remember, clinically
If by chance these are the pictures of MPA,
how would you distinguish this from
your GPA? Help me out.
#1: What kind of ANCA would
you find in MPA?
What kind of ANCA would you find
in GPA? PR3. Good.
Next, what if you were to do
a biopsy on this patient
and you did not find granuloma?
Then which one would it be?
If you did do a biopsy on a patient
and you did find granuloma,
then it would be GPA. Clear?
If not, make sure you come back
and review to this point
in your head mentally, distinguish
between the 2 conditions
of GPA and MPA.