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in this lecture we're going to discuss
lupus more formally known as systemic
lupus erythematosus or SLE lupus has a
unique pathology basically this is a
multi-system inflammatory autoimmune
disease that's caused by immune complex
mediated damage it affects multiple
organs in different ways in different
people and the cause is unknown there
are genetic factors environmental
factors and hormonal factors which
mitigate the disease it is more common
in girls than boys and the peak
pediatric age of diagnosis is 9 to 15
years of age we don't usually see it in
the very young children so the
pathophysiology of lupus is as we stated
both genetic and environmental in a
susceptible individual who has
susceptibility genes they're going to
have an event that results in a
apoptosis for example they might get a
sunburn on their skin a UV radiation a
period during which cells are breaking
down on a larger scale these patients
because of their genetic susceptibility
will have a defective clearance of these
a pop tonic bodies resulting in an
increased burden of nuclear antigens
throughout the body in this susceptible
person the B cells and T cells will
mount an immune response against these
nuclear antigens
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 these nuclear
antigen with antibody complexes are
causing the mainstay of the disease in
organs throughout the body so let's go
through some of the basic symptoms of
SLE or lupus constitutionally these
patients get frequent
fever's they may have fatigue and
they'll develop weight loss as well
their skin has a few key findings this
girl has one of them a malar rash they
may also have a discoid rash which are
little round circles around the skin
discoid rashes are also common in babies
with lupus and we'll talk about that
entity separately also patients may get
photosensitivity
which is a increased susceptibility to
burn when exposed to the Sun patients do
get lung involvement they can get
pleuritis pleural effusions pulmonary
hemorrhage or even pulmonary
hypertension they have cardiac findings
in additionally they will have
pericarditis myocarditis and
endocarditis 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 where there is
decreased blood flow to the distal end
of a digit in patients with lupus they
can get an endocarditis that is unique
called Libman sacs endocarditis
it looks grossly like bacteria under
kinetise but does not grow any organisms
on blood culture this can appear as
single or multiple one to three
millimeter warty deposits on those
valves and you can see a picture of one
here these patients may have involvement
of their renal pathways they may develop
protein area or hematuria depending on
if they're having more nephritic or
nephrotic syndrome they may develop a
pyuria
from just white blood cells that are
inflamed in the kidney but they will not
grow anything on the urine culture they
may develop hypertension as a result of
their end-stage renal disease and they
may proceed to renal failure 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 essentially any part
of the GI tract can be involved they may
have muscle this
orders they may develop arthritis or
Myositis or avascular necrosis of a
joint and importantly they may develop
brain problems roughly 10% of patients
with lupus will prevent present with a
brain problem
examples include stroke psychosis
seizure Korea or a tremor they may
develop a transverse myelitis of the
spine additionally patients can get
problems with their blood and any blood
line can be affected they may get
lymphopenia anemia or thrombocytopenia
any cell line on the CBC can go down so
this is a lot of stuff these are a lot
of symptoms to remember and
unfortunately there's no gene test for
lupus it's a clinical diagnosis for a
diagnosis you need four or more of the
eleven major criteria of lupus who can
remember eleven criteria of lupus well
you can with a little pneumonic here it
goes MD please offer all our ends a
holiday immediately again MD please
offer all our ends a holiday immediately
you have to do the S after the RNs let's
look at it this way
MD please offer all our ends a holiday
immediately what's clever about this is
the last three are lab criteria and the
first eight are body physical exam
criteria let's go through them again so
we can remember them M is the malar rash
D is the discoid rash and P is the
photosensitivity those are the skin
findings in lupus o is oral ulcers a is
arthritis the R is renal involvement and
the N is neurologic involvement the S is
tricky it's serous itis remember your
serosa are superficial coverings of your
organs so the area around the heart is
the pericardium that is a serosa so
pericarditis is a serous itis
peritonitis is a
zero situs pleuritis or a pleural
effusion is a serous itis next the labs
the a n/a test is its own criteria the
interesting thing about the a n/a test
is that virtually every patient with
lupus will have a positive a na this is
the one that almost all of them have
that is positive so a positive a na is
necessary almost for a diagnosis of
lupus if they're not any positive they
will be very soon
the challenge is is that one in three
people walking down the street will have
a positive a a heck they're even a
little bit infectious and by that I mean
if you are a May and a negative and you
move into the home of someone who is a
in a positive you're more likely to
become a a positive that doesn't mean
you're getting lupus so the a na
positivity is very is necessary but not
sufficient for the diagnosis H is heme
Labs again back to any of those elements
of the CBC they may have an abnormal
white count normal abnormal hemoglobin
or abnormal platelets and all of them
are usually low and I is immune labs and
I'll walk you through what those are in
a bit
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you