00:01
Depending again where you are
getting the immune complexes
or the antibodies accumulating,
you can have bowel
pain and bleeding.
00:09
You can have muscle pain,
you can have weakness.
00:12
And there's cutaneous
purpura which has been
demonstrated on the
image on the right.
00:18
Granulomatosis with polyangiitis,
which is a variant on this anca theme,
occurs more frequently in men,
peak incidence in the middle years.
00:28
Again,
the etiology is not understood.
00:32
But we know in this
particular case,
we are getting a greater activation
of T cells and macrophages,
it's not just
neutrophil activation.
00:43
And there are kind of three general
presentations, a necrotizing granulomas
and necrotizing
granulomatous vasculitis,
or focal necrotizing vasculitis,
all of these things can occur.
00:56
And the particular
variations on a theme,
why they have
variation is not clear.
01:04
We do know that there's a T cell
mediated hypersensitivity response,
and it's to
environmental antigens,
or to other microbial are
to microbial infection.
01:15
So the classic features of
Granulomatosis with polyangiitis,
because of the way that the
antigens are presumably getting in
either inhaled or say pulmonary
infectious is that more commonly
this entity is associated
with pneumonitis
and can have
cavitary infiltrates,
we can get destruction
of the lung parenchyma.
01:35
That's the majority.
01:37
They've also have a
chronic sinusitis.
01:39
So again, the inflammation
that's driving the vasculitis
tends to be in the nasal
and pulmonary passages.
01:50
You get mucosal
ulcerations of nasopharynx.
01:53
And because again,
you can also have these same anca lesions
occurring in the kidney,
you can have hypertension.
02:03
This is just showing you again in a
schematic way, what I've just mentioned.
02:07
And so, again, more commonly
we're going to see sinusitis,
mucosal alterations of the
nasopharynx and pneumonitis.
02:15
The lesions with polyangiitis are
a vascular intimal hyperplasia
a vasculitis with granulomas,
nothing too specific about that,
but it tends to involve
the smaller vessels.
02:27
The recommended therapy for
arthritis includes steroids
with either rituximab or
cyclophosphamide
Rituximab is an anti-CD20 monoclonal antibody
that will cause B lymphocyte killing.
02:39
That intern leads to reduced
antibody responses.
02:42
Cyclophosphamide on the other hand is an
alkylating immunosuppressant agent
Longer term maintenance therapy will involve
rituximab or other immunosuppression.
02:52
Finally, in the same category
of ANCA associated vasculitis,
Churg-Strauss
syndrome is the third.
03:00
The etiology like the
other two unknown.
03:03
The epidemiology is that
it's often associated
with a greater
eosinophilic activation.
03:08
So you're going to get
asthma, allergic rhinitis,
lung infiltrates,
and peripheral hypereosinophilia.
03:14
Signs and symptoms
of Churg-Strauss.
03:16
Again,
because of the hypereosinophilia
is that we tend patients
tend to have asthma attacks,
they have an allergic rhinitis,
they have the same cutaneous manifestations
as the other so a palpable purpura,
you get a mononeuritis
multiplex,
you can get involvement of the
nerves although less common,
and you can get inflammation of other
structures such as the pericardium.
03:39
In about 60% of patients for unclear
reasons, you can get a cardiomyopathy,
some of this may be
driven by cytokines,
some of this may be driven by vasculitis
involving the vessels of the heart.
03:50
And then gastrointestinal involvement
will manifest with bleeding.
03:56
One last category of vascular injury
vasculitis that we should talk about,
is immunoglobulin G4,
IgG4-related disease.
04:08
This is an entity that's
only been recognized
in perhaps the
last decade or so.
04:12
It's a disorder that's characterized
by high plasma levels of IgG4
so a particular isotype
of immunoglobulin G.
04:20
And there's an associated tissue
fibrosis that occurs frequently
with IgG4-expressing
plasma cells.
04:27
So that's the characteristic
histologic appearance fibrosis
with a lot of IgG4 plasma cells.
04:35
Interestingly,
depending on the disease,
it can affect just the pancreas
or just the biliary system
or just the salivary
glands or just the aorta,
or just pick your structure.
04:46
So we can affect any
variety of things.
04:49
The commonality is the fibrosis,
and the commonality is
the association with IgG4.
04:56
So the clinical presentation
depends on what vessel
or what structure
has been attacked.
05:01
And when we're talking about vasculitis,
it's going to tend to be the aorta,
and the periaortic structures.
05:09
In some cases, the damage that's
elicited by the inflammatory infiltrate
is such that it weakens the wall
sufficiently to cause aneurysm formation.
05:21
The mainstay of therapy like
most inflammatory vasculitis
is steroid therapy.
05:27
And with that we've
concluded a rather broad look
at the various vasculitis.
05:34
And hopefully,
you won't ever encounter one
because they can be quite
devastating in your patients,
but it's important
that you recognize them
because they want you to
recognize them on the boards.