00:01
Okay, let's look at a
different category here.
00:05
Let's look at
Thromboangiitis Obliterans.
00:09
The epidemiology on this one
is a very interesting disease,
it typically will happen
primarily in heavy smokers.
00:16
Typically heavy smokers who
are male and who are young.
00:21
So, an individual comes to your practice
and they smoke three packs a day,
and they suddenly have numbness
and tingling in an extremity,
which is what the vessels
that tend to be involved,
that's very likely to be a Buerger's
disease Thromboangiitis obliterans.
00:41
So we really don't have a
good understanding of etiology
there are certain
genetic predispositions
associated with
certain HLA types.
00:50
What we think is
happening in this disease
is that there are antigenic
components in cigarette smoke
that in a predisposed
individuals,
certain HLA types binds to the
endothelium and acts as a hapten.
01:03
That now that vessel wall gets
recognized as being foreign
due to whatever is in the cigarette
antigens that are binding to it.
01:11
We recruit and activate
in the predisposed individual
lymphocytes and macrophages
that then cause
endothelial cell damage
and we get thrombosis.
01:22
And so histologically
what this looks like
is this on the left hand side,
we have a vessel with
vasculitis going through it,
destruction of the media and an
organizing thrombus within the lumen
instead organizing thrombus
that can happen recurrently that is
associated with the loss of vascular flow.
01:46
And you can have more than just a
transient numbness and tingling,
you can have actually
loss of digits.
01:53
And as I say, Buerger's disease
or thromboangiitis obliterans
typically affects
the extremities.
01:58
It can affect other vessels,
but it's typically the extremities.
02:01
And we see actual gangrenous necrosis
of a middle digit of the right hand
and of multiple toes.
02:10
So, signs and symptoms
of Buerger's disease.
02:13
intermittent claudication of
feet, legs and hands and arms.
02:16
And again, it's on extremities
and it's because of the intermittent
formation of a thrombus.
02:20
So the patients lose
blood flow intermittently.
02:26
It can progress to
pain as we get necrosis
and eventually ulceration or
actual loss of the extremities.
02:34
Because we are damaging
nerves at the same time
that we are damaging the
actual extremity with necrosis,
you can get paresthesias,
you can get abnormal tingling sensation.
02:44
Okay, so that is an example,
kind of a medium
vessel vasculitis
that's associated with
binding to endothelial cells
that have been haptenated
by cigarette smoke
kind of a cool disease.
03:01
Alright, let's talk
about Kawasaki's disease.
03:04
So this is one of the most
common vasculitides of childhood,
kids don't get a lot of vasculitis
but when they do, it's often Kawasaki.
03:11
What is Kawasaki?
Boys are more commonly
affected than girls.
03:19
The vast majority of cases occur
in children under the age of five.
03:24
And a notable point.
03:26
A notable point is
that in adult cases,
it occurs in a quarter of adult
cases in patients with HIV.
03:36
So, how does it happen?
We have infectious agents
viruses most common
but you can also have this as a
result of a bacterial infection
that can then trigger the elaboration
of various autoantibodies,
and can trigger a delayed
type hypersensitivity
response against cross
reactive antigens.
03:54
So some infection,
the body responds to appropriately.
03:57
But now that's cross
reactive with self antigens.
04:02
The subsequent B cell
response making autoantibodies
can then cause damage
to the endothelial cells
and medial smooth muscle
cells just like we've seen
when we're talking about the
conceptual basis of vasculitis.
04:15
Classic signs and symptoms.
04:17
So the kids have fever,
they have a desquamative rash,
they have malaise as part of the fever
and systemic manifestations of cytokines.
04:27
They have very prominent erythema
of the lips and oral mucosa.
04:31
So, what are shown there is
a classic strawberry tongue.
04:37
They have cervical lymphadenopathy
due to the activation
of the little a type hypersensitivity
and B cell activation.
04:43
And they will have edema
the hands and feet,
again, because of the systemic
manifestations of cytokines
elaborate in very
various vascular beds.
04:52
So, the most feared complication
of Kawasaki's disease
is the involvement of
the coronary arteries.
04:59
And if we get inflammation
of coronary arteries
that can lead to
aneurysm formation
and over time that aneurysm
because of abnormal flow
through the dilated vessel
can cause a thrombosis.
05:10
And you can have a heart
attack in a 4-year-old kid.