00:01
In this lecture,
we're going to discuss lupus,
more formally known as
systemic lupus erythematosus or SLE.
00:11
Lupus has a unique pathology.
00:15
Basically, this is a multisystem
inflammatory autoimmune disease
that's caused by
immune complex mediated damage.
00:24
It affects multiple organs,
in different ways,
in different people.
00:28
And the cause is unknown.
00:30
There are genetic factors,
environmental factors,
and hormonal factors,
which mitigate the disease.
00:37
It is more common in girls
than boys.
00:41
And the peak pediatric age of
diagnosis is 9 to 15 years of age.
00:46
We don't usually see it
in the very young children.
00:50
So the pathophysiology of lupus is,
as we stated
both genetic and environmental.
00:56
In a susceptible individual
who has susceptibility genes,
they're going to have an event
that results in a apoptosis.
01:05
For example, they might have got
a sunburn on their skin,
a UV radiation.
01:10
A period during which cells are
breaking down on a larger scale.
01:16
These patients because of
their genetic susceptibility,
will have a defective clearance
of these apoptotic bodies,
resulting in an increase burden
of nuclear antigens
throughout the body.
01:29
In this susceptible person,
the B cells and T cells
will mount an immune response
against these nuclear antigens.
01:37
This will create anti-nuclear
antibody antigen complexes,
which will then go through the body
going to stimulate
both dendritic cells,
which then stimulate B cells
and create antibodies vastly
against nuclear antigens.
01:54
These nuclear antigen
with antibody complexes
are causing
the mainstay of the disease
in organs throughout the body.
02:02
So let's go through some of the
basic symptoms of SLE or lupus.
02:08
Constitutionally,
these patients get frequent fevers,
they may have fatigue, and they'll
develop weight loss as well.
02:16
Their skin has a few key findings.
02:18
This girl has one of them,
a malar rash.
02:22
They may also have a discoid rash,
which are little round circles
around the skin.
02:28
Discoid rashes are also common
in babies with lupus
and we'll talk about that
entity separately.
02:35
Also, patients may get
photo sensitivity,
which is a increased susceptibility
to burn when exposed to the sun.
02:44
Patients do get lung involvement.
02:46
They can get
pleuritis, pleural effusions,
pulmonary hemorrhage,
or even pulmonary hypertension.
02:52
They have cardiac findings.
02:55
In additionally, they will have
pericarditis, myocarditis,
and endocarditis.
03:00
Any part of the heart
can be evolved,
and they can get cardiovascular
or vascular problems
such as Raynaud's phenomenon,
which you can see
in this patient here.
03:09
Where there is decreased blood flow
to the distal end of a digit.
03:15
In patients with lupus, they can get
an endocarditis that is unique
called Libman-Sacks endocarditis.
03:22
It looks grossly like
bacterial endocarditis,
but does not grow
any organisms on blood culture.
03:29
This can appear as
single or multiple
1 to 3 millimeter warty deposits
on those valves
and you can see a picture
of one here.
03:39
These patients may have involvement
of their renal pathways.
03:43
They may develop
proteinuria or hematuria
depending on if they're having more
nephritic or nephrotic syndrome.
03:49
They may develop pyuria
from just white blood cells
that are inflamed in the kidney,
but they will not grow anything
on the urine culture.
03:57
They may develop hypertension
as a result of their
end stage renal disease
and they may proceed
to renal failure.
04:05
The GI system
can get involved as well
and in particular,
these patients may develop
oral ulcers, pancreatitis,
hepatitis, intestinal vasculitis
or even a
protein-losing enteropathy.
04:18
Essentially, any part
of the GI tract can be involved.
04:23
They may have muscle disorders,
they may develop
arthritis or myositis,
or a vascular necrosis of a joint.
04:31
And importantly,
they may develop brain problems.
04:34
Roughly 10% of patients with lupus
will present with a brain problem.
04:40
Examples include stroke, psychosis,
seizure, chorea or a tremor,
they may develop a
transverse myelitis of the spine.
04:52
Additionally, patients can
get problems with their blood
and any blood line can be affected.
04:57
They may get lymphopenia,
anemia, or thrombocytopenia
any cell line on the CBC
can go down.
05:05
So there
are several
ways that people can diagnose lupus,
and it gets confusing,
I think, for a lot of students because we
used to have 11 criteria and now there's
two different sets of criteria.
05:17
There's the ACR criteria
and the SLICC criteria.
05:20
The SLICC criteria are probably more
commonly used in children.
05:23
So I want to go through them here.
05:25
To meet criteria
you need four clinical criteria.
05:29
And then additionally one
immunologic criteria.
05:33
So we can go through them here.
05:35
And the clinical criteria include acute
cutaneous lupus, chronic cutaneous lupus,
oral or nasal ulcers, Non-scarring
alopecia, arthritis, Serositis.
05:47
Remember, the cirrhosis are the lining
of the pericardium,
the pleura and the peritoneum.
05:52
So any kind of inflation
of those services, renal,
any renal involvement,
any neurologic involvement.
05:59
Keep in mind that about 10% of patients
will present with a primary
neurologic condition.
06:04
Hemolytic anemia,
leukopenia and thrombocytopenia.
06:09
Additionally, patients
need to meet an immunologic criteria,
and those include a positive ANA,
a positive anti DNA,
a positive anti Smith antibody, positive
antiphospholipid antibody
lo complement levels
or also a direct Coomb's test,
which doesn't
count in the presence of hemolytic
anemia has to sort of be in addition.
06:32
Keep in mind
that the ANA is an interesting test.
06:35
It's commonly a false positive,
but virtually never a false negative.
06:40
So a patient doesn't have an ANA it's
very unlikely that they have lupus.
06:45
So, this is a lot of stuff.
06:48
These are a lot of
symptoms to remember.
06:51
And unfortunately,
there's no gene test for lupus.
06:55
it's a clinical diagnosis.
06:57
For a diagnosis, you need 4 or more
of the 11 major criteria of lupus.
07:04
Who can remember
11 criteria of lupus?
Well, you can
with a little pneumonic.
07:09
Here it goes.
07:10
M.D., please offer all RNs
a holiday immediately.
07:15
Again, M.D., please offer all RNs
a holiday immediately.
07:20
You have to do the S
after the RNs.
07:22
Let's look at it this way.
07:24
M.D., please offer all RNs
a holiday imediately.
07:29
What's clever about this
is the last three are lab criteria.
07:33
And the first eight are
body physical exam criteria.
07:38
Let's go through them again
so we can remember them.
07:41
M is the malar rash.
D is the discoid rash.
07:45
And P is the photosensitivity.
07:48
Those are the skin findings
in lupus.
07:51
O is oral ulcers.
07:54
A is arthritis.
07:56
The R is renal involvement,
and the N is neurologic involvement.
08:02
The S is tricky. It's Serositis.
08:05
Remember, your serosa
are superficial coverings
of your organs.
08:10
So the area around the heart is
the pericardium that is a serosa.
08:14
So pericarditis is a serositis.
08:17
Peritonitis is a serositis.
08:19
Pleuritis or a pleural effusion
is a serositis.
08:24
Next, the labs.
08:26
The ANA test is its own criteria.
08:29
The interesting thing
about the ANA test
is that virtually every
patient with lupus
will have a positive ANA.
08:36
This is the one that almost all
of them have that is positive.
08:39
So a positive ANA is necessary
almost for a diagnosis of lupus,
If they're not any positive,
they will be very soon.
08:50
The challenge is, is that one in
three people walking down the street
will have a positive ANA.
08:55
Hack,they're even a little bit
infectious.
08:58
And by that I mean,
if you are ANA negative
and you move into the home
of someone who has ANA positive,
you're more likely to become
ANA positive.
09:07
That doesn't mean
you're getting lupus.
09:09
So the ANA positivity is necessary
but not sufficient
for the diagnosis.
09:16
H is heme labs.
09:18
Again, back to any of
those elements of the CBC.
09:21
They may have an
abnormal white count,
abnormal hemoglobin,
or abnormal platelets
and all of them are usually low.
09:28
And I is immune labs.
09:31
And I'll walk you through
what those are in a bit.