00:01
Here we’ll take a look at non-ANCA
associated small vessel vasculitides.
00:04
We have now completed our discussion of
those important ANCA-associated vasculitides.
00:10
So what do we mean by non-ANCA? Well these
are pure immune involvement issues.
00:15
In other words, we’re talking
about an immune complex
that’s circulating throughout the body,
circulating throughout the body
causing damage to the endothelial cells
and, therefore, resulting in purpura as
being your major clinical manifestation.
00:34
What is the immunoglobulin that you find
with Henoch-Schönlein purpura?
Is it IgA, IgG or IgM? It is IgA.
00:46
Another name for Henoch-Schönlein
purpura is IgA vasculopathy,
which is what our topic is. It has nothing,
I repeat—has nothing to do with ANCA.
00:57
Non-ANCA associated.
00:59
Who is your patient, clinically,
walking through the door? A child.
01:03
Next, the momma says,
“my child has a rash.”
“Momma, tell me, where is this rash?”
“Oh, it’s on the butt of my child and
it’s on the lower extremities.”
Now that the child is brought in,
you as the clinician
are checking for the signs of this purpura.
01:23
When you feel the lower extremity of the
purpura, you can actually feel the bumps,
called palpable purpura.
This is anasarca purpura.
01:33
The palpable purpura, glomerulonephritis,
now stop there for one second,
let me make sure that I give you
the significance of that.
01:41
Would you then call this Buerger? When you
find—now please know this, be careful.
01:46
Some researches out there do not
clearly distinguish between
anasarca purpura and Buerger. Let me make sure
that I make it clear for you. Here we go.
01:56
So when you have palpable purpura
of the lower extremity of a child,
#1 differential anasarca purpura.
02:03
What is the immunoglobulin to be
affected or to be involved? IgA.
02:08
You do a biopsy of this rash,
you’re going to find IgA.
02:12
Another name for anasarca purpura
is IgA vasculopathy.
02:16
Is it possible that the immune complex may
then travel through the blood vessels
towards the kidney and cause ischemia and,
therefore, cause glomerulonephritis?
It could. Would you then call it Buerger?
No, you would not.
02:30
So what is Buerger? Buerger is
IgA nephropathy.
02:35
So that was a discussion that we
had back in nephrology
and so, therefore, in Buerger,
it’s strictly IgA nephropathy
in which IgA is then depositing
in the mesangium
without involvement of your
blood vessels causing purpura.
02:50
So it has to be a clear distinction.
02:52
Otherwise, it’s impossible for you
to choose the proper diagnosis.
02:59
So once you leave the kidney and you
find significant palpable purpura,
and then you think about anasarca purpura.
03:06
If that’s not clear, make sure that you
repeat what I just said and not
take this section in here
of Henoch-Schönlein
and also take a look at the section where
I have discussed the very same
with Buerger and Henoch in nephrology.
03:21
So now we have a child, lower extremity
palpable purpura, maybe glomerulonephritis
and the IgA could be found in the
mesangium there as well.
03:29
So on immunofluorescence, clinically,
the biopsy if required,
clinically indistinguishable between the 2.
03:37
You could have issues with mesentery so
therefore abdominal pain
and look for arthritis.
03:42
That’s your clinical manifestation.
03:45
Diagnosis, as I said, you will then do
a biopsy of the purpuric region
and you will find which immunoglobulin?
IgA. Commit that to memory.
03:55
Management: Self-limited, thank goodness,
and short course of steroids might then
be required for symptomatic relief.
04:05
Another condition is called cutaneous
leukocytoclastic angiitis.
04:10
Here the skin lesions,
purpura, are palpable.
04:14
Now most of the times when you have
non-ANCA type of issues,
it will be palpable purpura.
Let me give you a differential here.
04:21
Usually secondary to drug reactions.
04:24
That is what your focus will be on.
Cutaneous leukocytoclastic angiitis.
04:30
On immunofluorescence,
testing the skin biopsy,
you’ll find that leukocytoclastic
vasculitis
and your next step of management is
remove the defending agent
and a short course of corticosteroids.
04:45
I’d like to show you the hands of a patient
with leukocytoclastic vasculitis,
defending agent—the drugs,
then causing a reaction.
04:54
The next type of management would then
be to remove the defending agent
whether it be antibiotics, chemotherapy,
what have you,
and then the symptoms of the patient would
subside quite significantly.
05:07
We have another condition called
essential mixed cryoglobulinemia.
05:11
It is a non-ANCA-associated vasculitis.
What is this referring to?
Immediately, your patient, most likely,
history of hepatitis C.
05:20
Back in the ‘70s, there was an
epidemic of hepatitis C.
05:24
Nowadays, we don’t have it as much but there’s
still a large population of hepatitis C.
05:30
And with hepatitis C, we’re getting better,
and actually incredibly successful
at treating genotype 1.
05:37
And the drugs there that you must know from
pharmacology in recent years,
or actually in recent year is
ledipasvir and sofosbuvir.
05:46
The combination of the 2 could be given,
and within a 9-month period,
could completely cure your
patient of hepatitis C.
05:53
That’s something from pharmacology
that you want to keep in mind.
05:56
Now the clinical manifestation,
it’s an immune complex type of issue.
06:01
What do they mean complex?
Causing damage to blood vessels,
therefore, resulting in—
no surprise—palpable purpura.
06:08
May also cause damage to the
kidney—glomerulonephritis.
06:12
Will also have issues with the digits,
mononeuritis multiplex and
occasional hemoptysis.
06:17
Your focus here on hepatitis C
clinical manifestation.
06:21
Your diagnostic studies.
Looking for cryoglobulins.
06:25
The proteins that have now precipitated.
06:28
With the immune complex being activated,
your [Inaudible 00:06:29] levels will drop,
your C3 and C4.
06:33
Rheumatoid factor could be positive.
06:35
Hepatitis C particles usually present,
but sometimes are absent during the flares
as they are part of the cryocrit.
06:43
Management: Steroids. In cases
associated with hepatitis C,
these are some drugs that you want
to keep in mind such as interferons
and ribavirin, but as I told you, there are
newer drugs out on the market
and will be asked of you.
06:57
One will be ledipasvir and the other one
would be sofosbuvir.
07:02
If I was you, I would research
some of the newer drugs that
are out there for a cure,
specifically genotype 1 hepatitis C.
07:12
Severe cases—plasmapheresis is what
you’re thinking to save your organs.