All right, So Rheumatology Part two. We're going to go over a number
of topics polymocytis, dermato myocytis, reactive arthritis, rheumatoid arthritis, chilgrin syndrome,
sclerodermae, and lupus. So a number of stuff. I'll try to
keep it as brief as possible, just the high yield stuff. If you
do want to buy merchandise and support the podcast, I did leave a link
in the show notes to shirt smugs things like that if you want to support
the channel. I really appreciate that, and as always, thank you so
much for the really nice comments and the support. Let's go ahead and get
started. So we'll start with polymiocytis and dermato myocytis. But to group these
together because they're both idiopathic inflammatory myopathies a lot of overlap. Dermato Myocytis is
essentially just polymonocytus with the rash, So don't overcomplicate these because they can easily
become overwhelming if you try to memorize every detail. Let's just know the basics
for the exam and move on. So real quick overview of the patho both
polyendermato myocytis are autoimmune conditions. Polymiocytis involves CD eight T cells that have essentially
gone rogue and are attacking and destroying your muscle fibers. Dermatomocytis, you have
an unknown factor that activates your C three protein. This leads to a cascade
of events which ultimately results in attack and destruction of the muscle capillaries and small
arterials. So both poly and dermato myocytis are much more common in women,
so be looking for a female in the vignette. Pretty much every condition I'm
going to talk about today, as most of your immune conditions are are going
to be more common in female. So you'll notice that's the trend today.
It's we're going over these. Let's talk about clinical manifestations next as well as
the physical exam findings because there's a lot of high yield stuff here. So
first, muscle weakness proximal, progressive, symmetric, So muscle weakness is the
most common clinical manifestation of both poly and dermato myocytis. Classically, it's going
to be symmetric and involve the proximal muscle, so the hips and the shoulders
are going to be really common and It's the reason why in a vignette,
the patient they're normally going to mention is going to have difficulties climbing stairs,
getting up from a chair, raising their arms above their head. You'll commonly
see that in vignettes. Usually the muscle weakness will be progressive with gradual worsening
over a period of weeks to months. Real real quick recap. Remember,
main clinical menifestations for both is going to be muscle weakness. Muscle weakness will
be commonly proximal, so shoulders and hips progressive over weeks to months. And
symmetrics so both sides affected, all right. So for probably midocytis, muscle
weakness is really the only clinical manifestation you need to know. And dermato myocytis,
though, in addition to the muscle weakness, you also got some pretty
high old skin stuff, so let's talk about that next. So there's three
things you have to know for dermato myocytis gotron papules, heliotrope rash, and
photodistributed poiskyloderma. WHF, let's actually talk about what that means. So gotron
papules, they're violaceous papules that are often scally and ulcerated that often occur over
the bony prominences. Most commonly you'll see these over the dorsal aspects of the
metacarpo, philangeal and endophalangeal joints. So basically you got some scaly psoriasis looking
rash that's on the top of the hands, mainly over the knuckles. That's
gotron papules. Next heliotrope rash, so heliotrope rash or heliotrope eruption. This
is just a rap on the periorbital skin. So a rash around the eyes.
It's most common on the upper eyelid. Disremember a rash surrounding the eyes
for heliotrope rash. So gotron papules and heliotrope rash, those are pathenemonic features
of dermato myocytis. Anytime you see that they mentioned something is pathhenemonic for a
certain disease. That should mean to you this is going to be on the
exam. So if you see those two ever mentioned right away, be thinking
dermato myocytis, memorize those. There is one other finding that also seems to
come up on exams a lot. That's photo distributed pokloderma or the shaw sign
or the V sign. It's a complicated name to say, but very simple.
This is a rash or hyperpigmentation that shows up anywhere that's exposed to sunlight.
But classically and the way that it's going to be in the vignette and
the way they should learn it is on the upper back, neck, and
upper chest, which is why they sometimes call it the Shawl sign or the
V sign because that's kind of where those areas are distributed. So again for
dermato myocytis, remember gootron papules rash on the knuckles, heliotrope rash around the
eyes, and photo distributed poiklodrma rash around the upper chest and back. I'll
have a way for you to remember them at the end. Now for diagnosis,
you're going to start with labs. Specifically, first your muscle enzymes.
So there's a bunch of different muscle enzymes you can test for creating kinne s
though is the most sensitive muscle enzyme and it's the one most commonly tested for.
But also be on the lookout for your elevated aldolays. Even you can
even have an elevation of the muscle derived enzymes like LDHAST and ALT. Next,
we have your myocytus specific antibodies, anti Joe one and anti ME two.
There's actually a bunch of myocytis specific antibodies, but these are really the
only two that you'll you'll ever really hear about and the ones you should really
focus on. I have a couple way to remember these antibodies. One way
is to remember instead of dermato myocytis and polymyocytis, instead remember my Jocitus,
like M I JO citus, so polymydositius dermato mydositis and the m I and
the JO and my Joe. Situs helps you remember the anti joe an anti
ME two antibodies. So that's just kind of how I remembered it. So
instead of remembering poly myocytis, remember polymjocytis, am I JO. That can
kind of help you remember those two. And then I'll have another mnemonic at
the end that I'll go over. Anyways, these myocyte is specific antibodies,
they're far from perfect. They're really only positive in around twenty to forty percent
of people, and they also take weeks to come back. They're just one
of those labs that take a long time to get back, but they love
to ask about them on exam, so you have to know them. So
make sure you're thinking of poly or dermato myocytis. If you see these mentioned,
and specifically, if you see anti ME two mentioned, be thinking of
dermato myocytis. As anti ME two antibodies, they're highly specific for dermato myocytis.
And then you have your biopses, your muscle and skin biopsied. You're
not going to do these in every patients, but if you can't if you''re
not going to do it an every patient, but if you can't confirm the
diagnosis from clinical manifestations and lab results, then you canform a biopsied to confirm
the diagnosis and rule out your other differentials. Now there's other diagnostic tests that
I'm not going to go over, electromiography, skeletal muscle imaging for the exam,
there's four things you need to know for diagnosis, creating kinase, anti
JOE and ANTIME two and then your biopsy in that order, and you're done
for diagnosis. Let's talk about treatment next, because really there's only one magic
that you need to know, and that's going to be your glucocorticoids. So
steroids are your first line treatment. It's nice and easy. The steroids are
started at high doses for the first several months, then you slowly taper these
to the lowest effective dose normally for a total of around nine to twelve months.
You have some other agents that I wouldn't memorize, but hydroxyl chloroquin is
great to clear up skin manifestations intermato myocytis, but does nothing for the muscle
disease. And then you have some steroid sparing agents like methotrex site that can
be used as well. For the exam, high dose glucocorticoise is what you
need to know, all right. So between polymyocytis and or mato myocytis or
manto myocytis is the one you'll likely get tested on because it has those pathanemonic
findings we talked about before, as well as the more specific antime two antibodies.
So that's the one you should focus on. And here's how you're going
to remember everything that you need to know for dermato myocytis. So first on
the exam, they're not going to give you a forty nine year old female
that presents at the office today complaining of gotron papules, heuliotrope rash and photodistributed
poiculo derma. So it's not crucial to remember those ridiculous names because in the
vignette they're just going to say she presents with a rash or papules on her
hands around her eyelids. It's really important just to know where they're going to
have these dermatologic findings and then also remember the specific antime two antibodies. So
how do you remember this for the exam? Well, you remember for now
on dermato myocytis in your mind is going to be remembered as permatto myocytius.
All you're gonna do is to replace the D with the pep, and now
you have perm prm permatto myocytis. Why perm Because whenever you think of this
disease, I want you to think of a lady getting a PERM, sitting
in the chair. Her hair is in that little perm helmet thing, and
she's getting the works, she's getting her nails done, her eyebrows wax,
she's got that cape or a little shawl over her shoulders that you normally wear
in the salon or the arbershop and she's just relaxing and having some me time,
me time spelled with an m I. This is very hard without a
visual, so check out the YouTube channel if you need one, But basically
that's the visual you need to create in your head. Lady in a chair
getting her hair permed, getting her nails done, eyebrows wax, with the
cape or shawl that you wear in the salon, having some me time m
I time. Now, how does that help you remember what you need to
know? While she's getting her eyebrows wax, this helps you remember the heliotrope
rash that's common around the eyes, the upper eyele is especially, she's getting
her fingernails done. That helps you remember the gotron papules that are most common
on the top of the fingers. And she's wearing that cape or shawl.
That helps you remember the shawl sign or the photo distributed poikilo derma, which
is most common in the upper back, neck and upper chest. Exactly where
that cape is that you wear, exactly what's distributed anytime you get your hair
done and have that cape on, and then remember she's having some me time,
me time spell with an m I. That helps you remember the anti
me too antibodies, which are highly specific for dermato myocytis. So remember change
the D two A P. You have permatto myocytis. Lady getting a perm
having some me time, her eyebrows waxed, her nails done, and wearing
a cape, and that is dermato myocytis aka permato myocytis. Moving on to
reactive arthritis, This one's fairly simple. There's really just a few things to
know here. So reactive arthritis is just an arthritis that starts after an infection.
That's it. Now, what infection should you be looking for? This
is generally going to be a GI or a GU infection, so gastro intestinal
or genital urinary infection. So if you suspect reactive arthritis, make sure you're
looking for them. To mention in the vignette that the patient recently had a
bout of diarrhea or urethritis. So what specific bugs should we be looking for?
So when we're talking about our GI bugs or our enteric bacteria, there's
a bunch sell Manella, Shagela, campbelow, bactor C diff. Don't get
too hung up on memorizing those. But when it comes to your GU bugs,
your genital pathogens. There's really only one you need to know, and
that's clamidia Clamydia trachomatic. So if you only remember one infective organism that can
lead to a reactive authritis, definitely let it be chlamydia. This is the
one they'll often give you in the vignette, So remember that clinical manifestations.
So generally one to four weeks after infection, your clinical manifestations will start to
pop up. The immune system commonly targets the lining of the joint spaces,
which is why we get our arthritis, but can affect a number of other
areas as well that will go over. So first let's start with the peripheral
authritis. This is commonly going to be asymmetric olego authritis, mainly affecting the
lower extremities, especially the need some other MSK manifestations just to be familiar with.
They may have amphaesitis, which is an inflammation of the insertion site of
tendons. They may have dactylitis, which is an inflammation of the digits sometimes
refer to as sausage digits. This is going to be more common in your
patients with chlamydia infection and that's basically all you need to know if you're MSK
stuff. Mainly focus on the authritis. Next, let's talk about some of
the extra articular signs and symptoms. There's two that you need to be familiar
with. So first is your ocular symptoms, so inflammation of the eye,
whether it's conjunctivitis, anterior uveitis, keratitis, the ie, whatever part is
inflamed. And then we have our gu symptoms, so disuria, pelvic pain,
urethritis, servicitis, prostetitis. So the three things I just went over
form this triad. Triad consists of arthritis, conjunctivitis, and urethritis. This
is actually only going to occur in a small subset of patients, around a
third of all cases. There's definitely other clinical manifestations that can prevent present from
this condition or allusions cardiac manifestations, But for the exam, you damn well
better know that triad because they love to ask about it. So how do
you remember the triad? Well, you can remember this famous demonic It's not
mine, but it's a good one, and it's can't pee, can't see,
can't climb a tree. So they can't pee because of the genital urinary
symptoms, the urethritis, they can't see because of the ocular symptoms, the
conjunctivitis, and they can't climb a tree because of the authritis. So can't
pee, can't see, can't climb a tree, And you remember the clinical
manifestations you need to know for reactive arthritis. Now, clinical diagnosis is going
to be a clinical diagnosis. It's because there's no single definitive diagnostic test.
There's no validated diagnostic criteria. Even if you're getting cultures to check for an
underlying infection, often by the time the authritis presents, the pathogens may no
longer be retrievable. So the idea here this isn't something you need to memorize
for the exam, but you're basically looking for three things. One does this
patient have the characteristic authritis MSK finds we describe before asymmetric involvement a enthesitis dactylitis.
Two do they have evidence of a preceding infection diarrhea urethritis? And then
number three is there a lack of convincing evidence for another more likely diagnosis like
psoriatic authritis, rheumatoid athritis an fyi If they have joint effusion. You do
want to do an author a sentsis to rule out septic authritis, which can
present in a similar way. So again this is a clinical diagnosis. There's
nothing really to know for the exam in real life. Do they have the
typical MSK symptoms, do they have a preceding infection, and have you ruled
out other possible causes And that's it to make the diagnosis with one little exception
that I did want to mention because this may come up. So something known
as HLA B twenty seven. In case you hear about human leukocyte antigen B
twenty seven, know that this is found to be positive and around thirty to
fifty percent of patients with reactive authritis. So it's something that if it's positive,
can help support the diagnosis. But by no means does a negative test
rule out reactive authritis. So just a little fy in case that comes up.
So for treatment, there's not a lot to know here. Basically,
first treat the underlying infection. This is pretty straightforward. If they have chlamydia,
given them doxy etc. For the aarthritis, there is one main treatment
option that's agreed upon first line though that you should know and that's what you
should focus on. And that's end said. So nd sets are first line
treatment for authritis. So unless contraindicated, end sets are going to be what
you're going to use for the authritis. If they are refractory to the end
seids, you can use steroids. It's rare to require disease modifying agents like
methotrek state that we use in other types of authritis like RA because the symptoms
of most of these patients is generally self limited. So basically for the treatment,
focus on your end sets. That's the key takeaway here. So three
main high old things to know for reactive authritis. One look for your preceding
infection GI BUGG or chlamydia. Two remember can't pee, can't see, can't
climb a tree for your clinical manifestations. And three remember treat the underlying infection
if need, be an end sets for your authritis. And that's all you
need to know for reactive authritis. Let's talk about rheumatoid authritis next. So
this is a monster of a topic. There's a decent amount to know here.
Let's just focus on the need to know stuff. So rheumatoid authritis is
a chronic systemic inflammatory autoimmune disease of unknown etiology that can lead to the destruction
and deformity of joints due to erosion of cartilage and bone. Let's do a
thirty second path will breakdown on rheumatoid authortis, so RA, like so many
things in rheumatology, is an autoimmune disease. So we have these T cells
that are recruiting macrophages. They're all just hanging out in the joint space producing
cytokinds unnecessarily. This is leading to a bunch of inflammation. And then this
is the important part. These cytokinds that are being produced in the joint,
they're also causing the synovial cells to proliferate. So your synovial cells form your
synovial membrane. Remember that's your connective tissue that lines the joint space. So
synovial cells are proliferating and this causes this thick inflamed synovial membrane which is called
a panis. And this panis is growing in the joint eventually runs out of
space. The panis gets bigger and bigger, eventually starts breaking down and eroding
the joint space. And this leads to our clinical manifestations. So again cytokines
are causing this panis to grow bigger and bigger, eventually too big and it
just starts breaking stuff inside the joint. That's your thirty second path of explanation.
Next, who are you going to see this in? So that's going
to be females. So incidence of RA is twice as high among females compared
to males, So very likely for this to be a female in the vinet
number of other risk factors, lower socioeconomic status, cigarette smoking, obesity,
just really focus on the female sex. Though. Let's move on to clinical
manifestations because there's a lot of high yield stuff here. So there's a lot
of things to know for the clinical manifestations for r A. But luckily they
all start with an S, or at least I've created away from them to
all start with an S for the sake of memorization. So if you can
remember your seven s is for clinical manifestations for RA, you're good for the
exam, and of course I'll have a way for you to remember them at
the end. So the first S is symmetric, so symmetric joint involvement in
rheumatoid arthritis is a characteristic feature, meaning if the left hand is affected,
the right hand will likely be affected. To this, obviously in real life
isn't one hundred percent. It may be less apparent early on in the disease,
but you need to start doing is separating real life findings from exam questions
because they're two completely different things. You're studying for the exam, and an
exam is going to give you the most common presentation. So for an exam
question that they're going to describe symmetric joint involvement because that's the most common.
So count on that. Next S is going to be swollen. This is
an inflammatory athritis, so it makes sense the effected joints would be swollen,
tender, and warm. Next S is soft, So soft, warm,
boggy joints are typical of RA A. If they mention the joints are hard
and bony, this is not RA You should be thinking of osteoarthritis instead,
So remember soft boggy joints. Next s small joints. So rheumatoto athritis has
a predilection for the small joints hands, feet, wrist, so it's not
impossible, but less likely you'll see this in the shoulders, hips, spinus,
ceterup. So remember your small joints are more common to be affected hands,
feet, wrist. Next S spares the dip, so classically RA spares
the last knuckle, the distal interphalangeal joint, the DP. It's not impossible
for the dip to be effected, but if you see DP involvement, this
is more likely to suggest a diagnosis of osteoarthritis rather than r A. So
remember ra A classically spares the dip. Next s sixty minutes or more sixty
minutes or more so in all types of inflammatory polyarthritis like rheumatoid athritis. You're
looking for morning stiffness for extended periods of time. Depending on the literature,
some are going to say over thirty minutes, others are going to say over
sixty minutes. But the key here is that the morning stiffness RA is sustained,
and that's what's going to help you differentiate from osteoarthritis, where the morning
stiffness and osteoarthritis, if it's present at all, is usually transient or only
last for a few minutes at most. Last s swan neck deformity. So
there's a number of different joint deformities that are can cause botneer, swan neck,
boastring deformity. We don't see them as often as we once did due
to the early innovation of d MARDs, but I do remember Swaneck deformity coming
up on an exam question. So if you see this mention, this is
typically seen in advanced RA and commonly caused from the erosion of the extensor tendon
from chronic sinovitis. It causes the dip joint to flex and the pip joint
to hyper extend, so the middle knuckle is hyper extended, last knuckle is
flexed, and it kind of looks like a swan neck. So that's your
last s, all right. So for clinical manifestations and physical exam findings,
remember your seven s is some metric swollen, soft small joints, spares,
the d I P sixty minutes, and swan neck deformity. If you need
help remembering those seven ses, I got you. Here's the second part of
the mnemonic. So I used to remember rheumatoid authritis like with seven ses at
the end. So rheumatoid authritis, and when you say that out loud,
what do you sound like a snake? So every time you see rheumatoid authritis
on an exam question in school, say it like that in your head,
not out loud, and then picture a snake. But this is not just
any snake. It's a stuffed animal snake, so like a little plush toy
snake. Stuffed animal snake is obviously soft. That helps remember the joints are
soft and boggy. Next, this snake oddly has hands and feet. That's
very weird. Snakes don't normally have that, but this helps remember that the
ri commonly affects the small joints of the hands and feet. Next, the
snake is holding an identical timer in each hand and each time is set at
exactly sixty minutes. So the identical timer in each hand helps you remember the
symmetric findings because timers are identical, and the fact that the timers are set
at sixty minutes helps you remember the morning stiffness for you know, sixty minutes
or more over sixty minutes. Next, his stomach is super swollen, like
most snakes when they eat too much, you know they have that like big
swollen belly and the swollen stomach helps you remember the joints will be swollen in
ra a and what did he eat that made his stomach so swollen? Well,
if we look inside his stomach, we see two things, a swan
and a bowl of asparagus dip. That helps. The swan helps you to
remember the swan neck deformity, and the asparagus dip he ate helps you remember
spares. The DP A spare a guest stip d ip A spare I guess
d ip dip. So that visual combined helps remember the seven ss of hematoid
athritis. So quickly recap you have a soft plush snake helps remember soft joints.
He's got human hands and feet. Affects the small joints of the hand
and feet. He's holding an identical timer in each hand set to sixty minutes
symmetric and morning stiffness over sixty minutes. It's got a swollen stomach, swollen
joints because he ate a swan and asparagus dip swaneckity spares the dip. For
my podcast listeners, definitely check out the YouTube channel. I made an amazingly
horrible snake and Microsoft Paint then helps paint this visual all right, So next
let's talk about diagnosis. There's a number of different tests and labs for diagnosis
of r A, but there's two that you absolutely need to know, and
that's your rheumatoid factor and your anti CCP. So let's start with the rheumatoid
factor. So rheumatoid factor will be positive in seventy to eighty percent of patients
with URA, so it's a very sensitive test. Problem is, it's not
very specific. Even healthy individuals can be positive for rheumatoid factor. Patients with
lupus hepatitis, see a bunch of other inflammatory conditions can all wind up with
a positive rheumatoid factor. So it's a sensitive test. It's just not very
specific. So if we want a specific test, that's when we use our
anti CCP also known as our anti citrullinated peptide antibodies or anti sickl citrulinated peptide.
That's why we just call it our anti CCP. So this test is
very specific rheumatoid author rights, meaning if it's positive, this is very likely
going to be RA and not something else. The specificity is generally over ninety
percent, some literature stating as high as ninety five to ninety eight percent specific
for ra AW. I'm not going to get into the necessary details, but
both of these can actually be negative, the rheumatoid factor and anti CCP.
That can both be negative and you can actually still make the diagnosis of RAA.
It's called zero negative ra Just a little extra knowledge, not really anything
to know for the exam. So in conclusion, know your rheumatoid factor,
know your anti CCP. And a little quick tip. If you get an
exam question and they say which are the following is most specific for the diagnosis
diagnosis of rheumatoid authoritis, and you've narrowed it down to your rheumatoid factor and
your anti CCP, and you can't remember which one it is, remember the
words specific in it has two c's in it, and so does anti CCP.
At least that help me on the exam. So two things again,
and know for diagnosis rheumatoid factor and anti CCP. That's the high yield stuff.
There's obviously other immodalities you can use in diagnosis X ray ultrasound, but
for the exams, they're like going to mention your crologies and that's what you
need to know. Let's talk about treatment next. Now, two things you
need to know for treatment, and that's end sets and DMARDs. So end
sets, plain and simple, they're just for symptom control. If the patient
has contraindications to end sets or inadequate response to end sets, you can give
them corticoids. Like can really anything else. Not much to know here except
for the fact that end sets they're only going to treat the inflammation and pain.
They don't do anything to alter the course of the disease or to prevent
the destruction it can cause. And that's why your next class of meds is
the most important for rheumatoid athritis, and that's your DMARDs, so disease modifying
anti rheumatic drugs. There's a few meds in this class, hydroxy chloroquin soulfasalazine,
but if you're going to memorize one, make sure it's methotrex sate.
Metho trek sate is the most commonly used DMARD because compared to the other meds,
it has the faster on set of action, greater efficacy, better long
term tolerance. So just remember methotrex sate and you should be good for the
exam, let's talk about why it's so important to start patients on this class
of meds in the first place. So patients diagnosed with RA should be started
on these drugs as soon as possible because with R, once the damage is
done, it's irreversible. There's no going back. So starting this class early
on in the diagnosis can reduce or prevent this irreversible joint damage. So remember,
unless there's a contraindication, everybody with RA will get a d MART and
that DMART will likely be methotrex SATEN. So for an exam standpoint for treatment,
remember two things N sets for symptoms and dmarts to prevent disease progression.
In real life, obviously it's a little bit more complicated in that than that.
There's other options, but for the exam, that's what you need to
know, all right. So for rumatoid authoritis, there's a lot of info.
What do you absolutely need to know? Remember your seven S is for
clinical manifestations, symmetric soft, swollen small joints, sixty minutes or more,
spare the dip and swan neck. Remember your rheumatoid factor in your anti CCP
for diagnosis. Anti CCP is the specific one. Remember two seasons, specific
and anti CCP. And the main takeaway with treatment is your dmarts specifically methotrek
sate and that is a rheumatoid authoritis. Movie on to Schogrin syndrome, So
there's just a few things to know here. This is a systemic autoimmune disease
characterized by lymphocydic infiltration of exocrine glands. So there's a problem with the X
screen glands. There's this autoimmune induced inflammation of the exocrine glands, especially the
lachrymal and the salivary glands. This leads to diminished function of these glands with
result in dryness of the eyes and the mouth, among other problems. You
have primary and secondary types of this condition. Primary isn't associated with any other
conditions where secondary is associated with another underlying rheumatic disease like rheumatoidatis, lupus,
etc. Your focus should be on the primary type. So clinical menifestations,
there's three things you need to know when it comes to Schogrin syndrome. That's
dry eyes, dry mouth, parodic glands, swelling. Those are the most
common ones you need to know that will definitely be in the vignette. In
real life, the disease spectrum is much more broad. It's a systemic disorder
and you can have extraglandular organ involvement of the skin joints, heart GI tract.
But for the exam, all you need to rememorize is dry eyes,
which is also known as corrado conjunctivite. A SICA patient may complain of irritation,
greediness, itching, of foreign sensation in the eye, so look out
for that. Next dry mouth, which is also known as zerostomia. By
the way, these two symptoms, dry eye and dry mouth will be the
most common. Dry eyes and dry mouth are president and over eighty patients with
primary schogrin syndrome, and then the last one parodi swelling. So other salivary
glands can be involved, like the submandibular, but the product is going to
be the most common. So again for the exam, if you see dry
eyes, dry mouth, product gland swelling, be thinking schogrin syndrome next diagnosis.
So for diagnosis, there is no one single best test to diagnosis condition.
Make the diagnosis based on the appropriate clinical features combined with laboratory testing,
but no one specific test rules in or out the disease. What that kept
in mind, let's talk about the two diagnostic tests they will likely ask you
about on the exam. First one is your anti ROW and anti law antibodies.
Anti ROW also known as SSA and anti LAW also known as SSB.
This is probably the one you're going to be tested on. Anywhere from sixty
to eighty percent of patients with primary schogrins will test positive for one or both
of these antibodies. The problem is these can also be positive in patients with
lupus, even some healthy individuals without cholgrins. So it's a good test,
but it's obviously not perfect. Go usually order these antibodies in conjunction with your
ANA and your rheumatoid factor. And then the other diagnostic test that you should
know one that's often tested on is the Schermer test. So the Schermer test
is quite literally taking a dry piece of sterile paper and putting it in someone's
eye you suspect to shogrins, and then you measure how what the paper gets
over a period of five minutes. That's really it. It's a simple idea,
but it works well and you should know it. For the exam,
and then just a final note, if there's still any lingering doubt about the
diagnosis after doing your lab's physical exam, etc. You can use a salvary
gland biopsy to establish for the diagnosis. So outside of the clinical manifestations,
really the highest yield thing to know for schogrins are the diagnostic tests that went
over. You need to remember that. The way that I used to remember
anti row and anti law and the Shermer test is by instead of remembering Shogrin
syndrome, I would remember slowgreen syndrome. So instead of shogrin slow green slow
green syndrome, so slow green instead of shogreen, and what slow and green?
A frog? That to remember a slow green frog landed in my cup
of Sherbert. So create that visual in your head. You have a cup
of Sherbert, that little frozen fruit treat and a frog landed right in it.
So shogreen is now slow green and a slow green frog landed in my
cup of Sherbert. So frog. The second two letters are ro o that
helps remember anti row landed. First two letters La helps remember anti law,
and then Sherbert helps you remember the Shechrmer test. So that worked for me.
Just remember slow green instead of shogreen, and remember that slow green frog
landing in your cup of Sherbert. Remember second two letters of frog r O,
first two letters of landing R l A, and then Sherbert helps remember
schermer remember that visual. So for treatment, it's not very much to know
here. If their eyes are dry, give them artificial tears for dry mouth,
make sure they're staying hydrated, stop smoking. They can use salive vary
stimulants like sugar free candies or lozenges. That stuffs usually not test it on.
But what they will test you on is the muscarinic agonis or your cholinergic
specifically pilocarpine and sevemoline. These met stimulate secretion of the extracrine glands, opposite
of your anticholinergics like atropine and atrovent that most of us are more familiar with
that can dry you out. So if they ask you what meant to put
a patient on the shogrins, remember your coolinergics, pilocarpene, sevemoline. All
right, So that is Schhogrin's just a few takeaways here. It's an autoimmune
disorder of the exocrine glands. So remember your dry eyes, dry mouth,
prodig gland enlargement, remember your row in law and your schermer tests for diagnosis
and then treatment. Just remember pilocarpine and sevemoline and you're good to go.
Talk about systemic sclerosis or scleroderma next. So this is a systemic connective tissue
disease or excessive collagen deposition leads to progressive fibrosis of the skin and internal organs.
So the path that is not completely understood, But what we do know
is there is a genetic predisposition combined with some sort of triggering event like a
viral infection, drugs, etc. This is followed by an endothelial injury.
Our T cells come in start overproducing cytokinds, causing excessive inflammation. The cytokinds
activate fibroblasts and the fibroblast this is the important part, start depositing collagen and
because of this, our nice, soft and squishy skin and organs are now
being replaced with this hardened fibrotic tissue. And this fibrosis also causes reduced blood
flow and result in eschemic tissue damage. And this combination is what leads to
our clinical manifestations. So in conclusion, two damn much collagen and fibrosis.
So this is going to be much more likely in a female. In the
female population, so the female to male ratio can be as high as eight
to one, like pretty much everything we're talking about today, so much more
common in women. There's a few different types. Systemic sclerosis is generally classified
based on the extent of skin involvement and organ involvement. There's really only two
main types that you need to know. The first is limited cutaneous systemic sclerosis
aka Crest syndrome. So for limited disease, I just want you to remember
two things. One, it normally spares the trunk that's important, mostly affects
the distal limbs. And then the second part, and probably the most important
thing to know, is this limited form of the disease is associated with something
called Crest syndrome. So what is Crest syndrome? What? Luckily, Crest
actually comes packaged with its own pneumonic that explains exactly what it is. So
CREST actually stands for the C stands for calcinosis. Cutis are stands for raynod
phenomenon. The esophageal dismotility. The S stands for sclerodactylly, and then the
T stands for telangiectagium. So let's talk about what of these actually mean.
So, calcinosis cutis is a calcium deposition in the skin, so you just
get these clumps of calcium to it's all over raynot phenomenon. Is the same
thing that happens when your fingertips get really cold and vaso constrict and they turn
white and blue. This is just an exaggerated form of this, and it
can also happen in response to stress esophageal dismotility. This one's pretty straightforward.
They may have dysphasia, choking gird, etc. Sclerodactyle is this claw like
appearance of the hand due to the tightening of the skin of the fingers,
and Sclerodactyle sounds very much like pterodactyl, which is that big bird dinosaur bird
with the claude feet, So pterodactyl claude feet, sclerodactyle clawde hand, so
that kind of helped me remember it. And then finally tlangiac taja, which
is in so many other conditions so probably not the first time you're hearing this
term. But all this is these dilated superficial vessels that create this weblike appearance
on the skin. So that's your limited form of the disease. Again,
remember two things. One, this mainly occurs in the distal limbs but generally
spares the trunk. And then to remember crest syndrome, calcinosis, raynats,
esophageal sclerodactyly and telangiac taste. Moving on to diffuse cutaneous systemic sclerosis, so
the word diffuse is in the name, so as you'd imagine, this is
more widespread. So the areas we discussed before that limited sclerosis didn't really affect
like the trunk for instance, and proximal limbs, diffuse will affect this area.
This, of course isn't one hundred percent in real life, but for
the exam definitely learn it that way. Biggest takeaway with diffuse cutaneous systemic sclerosis
is that it involves the organs. That's what you need to remember. These
patients are much more likely to have involvement of the internal organs, especially the
heart, lungs, and kidneys, so look for lung fibrosis, scleroderma,
renal crisis. That's the key here. So recap. For diffused disease,
remember trunk involvement and organ involvement heart, lung and kidneys. For limited disease,
remember the distal limbs and spares the trunk, and then of course crest
syndrome one last time, even more condensed diffuse trunk and organs, limited,
distal limbs and crest done. That's it. Don't make it more complicated,
and it has to be. Let's move on to diagnosis. So for diagnosis,
there's really two labs you need to know. The first is your anti
centromere antibody. So the presence of this antibody is most often associated with the
limited subtype. Only about five percent of patients with this antibody will have diffused
disease. So if they give you a positive anti centromere antibody on an exam
question and they ask you which is the most likely diagnosis is one hundred percent
on an exam question, limited disease aka Crest syndrome. And that's why instead
of remembering this antibody is anti centromere, you're going to remember it as anti
crustomere. So no longer anti centromere and is now known as anti crustomere.
Just replace it in your head and you'll get the question right. Next anibody
is your anti topoisomerase, also known as your anti SCL seventy antibody. This
one you need to know is most commonly associated with diffused disease, and it
also carries a higher risk of severe interstitial lung disease if positive. So remember
anti crustomere associated with limited disease, anti topoisomerase SCL seventy anibody associated with diffused
disease. There's a couple other labs you can use, of course, your
ANA, your anti nuclear anibody, which is a very sensitive test and it'll
be positive and approximately ninety five patients, but it's just not specific for anything.
You can be positive in so many other conditions. Your anti RNA polymerase
three anibody. For the exam though, focus on your anti centromere and aka
anti crustomere and your anti topoisomerase. That's what they're going to test you on,
all right. So for treatment, it's unlikely they're gonna ask you about
treatment because treatment is mainly targeted at treating the symptoms or organ that's involved.
So if they If they have GIRD, you're going to give them a PPI.
If they have a NO phenomenon, give them a calcium channel block calcium
channel blocker. Renal problems, give them an ase inhibitor. Patients with severe
inflammatory organ involvement or diffuse skin involvement are usually treated more aggressively with systemic immunal
suppressive therapy like methotrech sate. There's no first line agent and certainly nothing I
feel is necessary to memorize for the exam. They're going to ask you a
question. It will be about the clinical manifestations or the antibodies that went over
in diagnosis, so focus on those, so key takeaway here. First,
this will be a female and divignette. Know your two main types limited,
crest and diffuse. Know that limited involves mainly the distal limbs and your crest
findings, but spares the trunk. No that diffuse involves the trunk and your
organs. No anti centromere aka anti crustomere is associated with limited or crest disease,
and then anti topoisomerase is more likely to be associated with diffused disease.
Treatment again is organ symptoms specific done. Last topic is going to be systemic
lupus erthematosis. Another really big topic, but let's distill it down as best
we can. So this is a multi system immune mediated disorder characterized by antibodies
to nuclear and cytoplasmic antigens. So the specific cause of SL is unknown,
but we do know that many of the clinical minifiztations that we see are caused
by antibody formation and the creation of immune complexes. So these complexes deposit all
over the body, the heart, kidneys, skint, They cause a number
of problems inflammation, tissue damage, cell death. This whole process, by
the way, is a type three hypersensitivity reaction. Surprise, surprise, This
is much more common in women, especially women of childbearing years, so look
for a younger female in the vignette. So a couple of reasons why,
which I'm not going to get into, but it has to do with estrogen.
Also some factors related to the X chromosome which seem to play a role.
This is also going to be more common in African American patients in the
US. African American women are two to four times more likely to have SLEE
than Caucasian women. So the vignette I would be looking for an African American
female. Clinical manifestations literally everything you can think of, hypertension, arthritis,
alopecial and patternopathy, dysphasia, thrombosis, fever, weight loss, fatigue.
Lupus is the great imitator. It affects almost every organ. Therefore, it
can present like so many different diseases. There's a ton of clinical manifestations and
it would be very unwise to memory rise them all. Let's just talk about
the ones they'll likely test you on and the more common one. So first,
you have your constitutional symptoms, fatigue, fever, weight loss. These
are all very non specific, but almost every patient with sl will have these
symptoms at some point during the course of their disease, so they're very common.
They're just not very specific. Next, rash and a melar distribution aka
your butterfly rash, sparing the nasal labial folds, ring the alarms. This
is the thing to know when we're talking about clinical manifizations for lupus. If
you can only pick one thing to know for the exam for clinical manifizations,
this is going to be it. So a melar rash can occur in other
conditions, but if you see it on an exam question, especially if they
see it spares the nasal labial folds. It is lupus, so it's the
most common skin lesion to have in lupus, and it presents as erithema and
a melar distribution over the cheeks and nose, sparing the nasal labial folds.
It normally appears after sun exposure, or almost always appears after sun exposure.
So you have this photosensitive rash that shows up. And remember that word photosensitivity
as it's really important because many of the skin manifestations in lupus appear after sun
exposure. The rash looks like a butterfly over the nose and cheeks, and
it's in a melar distribution. In case you're not familiar with the term,
melard is just another name for the zygomatic bone of the face, which are
your cheekbones. So basically malar distribution just means over the cheekbone area. Remember
this rash spares the nasolabial folds, which are the little creases from the corner
of your nose to the corners of your mouth, and this gives it that
butterfly wing appearance. So remember rash over the cheeks and nose, sparing the
nasolabial folds after sun exposure. Remember that rash, memorize it, know it
well. It will come up next your discoid lesions. While we're on the
topic of skin manifestations, the next one that you should know is your discoid
lesions or discoid rash or discoid lupus, however you see it come up.
These are these round or coin shaped erathematose raised patches that often cause this pretty
dramatic atroph scarring. Just an fyi. If you know who the singer Seal
is, who's really big in the nineties, He's sang that song Kiss from
Arose. The scars in his face are actually from discoid lupus legions. So
maybe I'll help you remember and to associate those two. Authritis. So authritis
and arthrologists are going to be seen and around ninety percent of patients with slee
it's often one of the earliest manifestations and a lot of times it's the reason
why these patients come into the office to be seen to begin with. And
then we have our cardiovascular findings. So pericarditis is going to be the most
common cardio complication of sl so that'd be the one to focus on, but
they can also have issues related to endocarditis, higher risk of myocardial in function,
so just remember the increased risk of cardiovascular problems in these patients. Next,
renal, So around fifty percent of patients will have some sort of renal
manifestation, whether it's the materia, proteinuria, nephrodic syndrome, glomarulan nephritis,
secondary hypertension. Look for problems with the kidneys, and patients with sl thrombot
embolic disease. So throwmbot embolic disease is a potential complication of SLEE, particularly
in the context of antiphospholipid antibodies. The tenure risk of a thrombotic event for
a patient with SLS actually around thirty three percent. And then we have our
hematologic abnormality, so all three blood cell lines can be affected in patients with
sl so your red blood cells, whitelod cells, platelets, so I'll be
looking for anemia, leukopenia, thrombocytopenia. So even though I just went over
a ton of clinical manifestations, there's a bunch that I didn't even touch on,
and I'm not going to neurologic problems like strokes, seizure, GI problems
like aesophagitis, pancreatitis, pulmonary issues like pleuritis, pneumonitis, oral ulcers.
The list just goes on and on. But the ones I went over,
those are the ones that I'd focus on. Those are the ones that often
come up on exam questions. And the way if you want to remember those
is by remembering the mnemonic MD chart. So MD chart an MD chart like
doctor medical doctor chart, like a doctor's chart that stands for The M stands
for me lar rash, the D stands for discoid lesions, and then the
chart part of it. The C stands for both constitutional and cardiovascular manifestations.
The H stands for hematologic abnormalities, the A stands for arthritis, the R
stands for renal, and then thromboembolic disease is what the T stands for.
So just remember, if you want to remember some of the main clinical manifestations,
remember m D chart, and that's how you remember some of the main
ones. So for diagnosis of lupus, like so many things in rheumatology,
it's a complicated one. It's not often based off of any single lab result
or imaging study, but rather recognizing this spectrum of clinical manifestations accompanied by supportive
serologic studies and excluding a number of alternative diagnoses. With that being said,
from an exam standpoint, it's a bit easier as it often is. There's
really just a few labs that you need to know. So first let's start
with your ANA, your anti nuclear antibodies. I've mentioned this and some of
the other ones too, but this is your screening test. This is where
you start your anti nuclear antibodies ANA. Because this test is very sensitive,
it's positive and virtually all patients with sl The problem is it's not very specific.
It's positive in a number of other conditions. The reason you get this
test is because if it's negative, well not to say that it's impossible for
the still to be lupus, but much much less likely. So screen with
your ANA if they have lupus, around ninety five percent chance this is going
to be positive. And then you move on to your more specific labs,
the ones that you really need to know. And first that's going to be
your anti ds DNA or anti double stranded DNA and then your anti Smith antibodies.
Those are the two that you really need to focus on for lupus.
They're both highly specific for lupus. So remember that Smith and double stranded DNA
associate those two very important. So antids DNA anti double stranded DNA anti Smith
antibodies very specific for lupas. You need to know those. And then I
have a mnemonic for those two. This is definitely a weird one, but
sometimes the more weird it is, the easier it's to remember. So the
word lupus sounds like the name Lu, like Lulu and piss like taking a
piss being so I used to remember a guy named Lou taking a piss on
his Smith and Wesson double barrel shotgun. Not sure why he's peeing on a
shotgun. Maybe he's very anti gun. Anyway, Smith and Wesson is the
famous gun manufacturer and a double barre shotgun as well, a double barrel shotgun.
So Smith and Wesson helped me remember the anti Smith antibodies, and double
barrel shotgun helped him me remember the anti double stranded DNA antibodies. So remember
when you see lupus, I want you to think of a guy named lou
taking a piss on a Smith and Wesson double barrel shotgun. Create that visual
in your head and you'll remember the two main labs you need to know for
lupus, anti SMITH and anti double stranded DNA. One other lab I wanted
to really quickly mention that you should be on the lookout for is your anti
fospiphul lipid antibodies. Forty percent of patients with sl will be positive for anti
FOSPIHL lipid antibodies, which is associated with anti fospiphil lipid syndrome, and patients
with the syndrome matter of high risk for venus arterial thrombosis miscarriages. So I
just wanted to mention that in case it does come up treatment. So there
is a lot to know overall for lupus. But the good thing is for
meds, there's really only one medication that you should focus on for the exam,
and that's hydroxy chloroquin. So all patients with lupus need to be on
hydroxychloroquin lupus. Hydroxy chloroquin lupus, hydroxy chloroquin, hydroxychloroquine lupus. Associate the
two in your brain forever. This medication improves constitutional and MSK symptoms, it
improves the mucocutaneous manifestations. It even has a positive impact on patient survival.
So remember this medication for lupus and then also be aware of your steroids and
end said. Some immunosuppressive agents like microphenolate can also be used for treatment and
combination with hydroxychloroquin. Steroids are especially helpful in severe or life threatening cases where
you can use these high dose ivy pulses of methylpredness owe to control the disease
and all tissue injury. But the main takeaway here is just to remember hydroxychloroquin
so lupus triple distilled. This will be a female in the vignette. She
will likely present with the MD chart manifestations we went over screen with your ana.
Then remember Lou took a piss on a Smith and West and double barrerow
shotgun, anti smith and anti double stranded DNA. Those are the specific ones
treatment. Remember hydroxy chloroquin and that is lupus. Let's wrap it up with
five quick questions. Question one, forty two year old female presents the office
complaining of heartburn, small white lumps on her fingers as well as a tight
feeling in her hands that makes it difficult to make a fist on physical exam,
you knowe tolange ectasias on the palms and face. Labs are positive for
both anti nuclear antibodies as well as anti centromere antibodies. What diagnosis should be
suspected in this patient? So that would be limited systemic sclerosis aka CREST.
So this patient has a very classic presentation and presents with a number of the
manifestations of CREST syndrome. So we see the calcinosis cutus, those small white
calcium deposits in her hands. She has heartburn which is from the esophageal this
motility disorder tolange ectasia's as well as the tightening of the skin of the hands.
This is a classic limited systemic sclerosis which we know will generally have a
positive a NA and then most importantly a positive anti centromere and a body test
aka anti Crestomere. Two, a forty seven year old female presents to the
office complaining of dry mouth and dry eyes for several months. She has used
over the counter eye drops with minimal improvement. Physical exam reveals dry mucous membranes
and swollen swollen, parodied glands. You explain to the patient you will be
performing a test to assess for tear production. What is the name of the
test that will be performed? So that is going to be the Schermer test.
So this patient very likely has Schoburn syndrome. Of course we would also
need to perform some labs anti row, anti law NA, but she has
all of the classic clinical manifestation's dry eyes, dry mouth, product land enlargement.
And the test we've performed to assess for tear production is the one called
the Schermer test. Question three. A fifty one year old female presents to
the office today complaining of muscle weakness. She describes difficulty combing her hair rising
from a chair. A physical exam, you know, a rash around the
eyes and the eyelids, violaceous papules of the dural dorsal aspect of both hands,
as well as erathema across the shoulders, upper back, and upper chest.
Labs are drawn which show an elevated creating kinase level as well as a
positive antime two antibodies. What treatment should be initiated in this patient for the
suspected diagnosis. So that is going to be glucocorticoids. So this patient has
dermato monocytis, She has the gotron papule's heliotrope rash, decreased muscle strength,
the Shaw sign, plus elevated CK and antimeto antibodies. That's about as clear
cut as you can get, and then we know for dermato myocytis. Glucocorticoids
are the cornerstone of your initial therapy. This is usually pregnos own added dose
of one miligram per kilogram per day. Question four. A forty one year
old female has symptoms consistent with rheumatoid athritis and labs are drawn to assist in
making the diagnosis. The physician assistant informs the patient that a rheumatoid factor as
well as a very specific antibody for rheumatoid arthritis are both elevated. Which antibody
specific to rheumatoid arthritis is likely elevated in this patient. So that is going
to be your anti citrilinated anti citrilinated peptide antibodies, aka your anti ccpanibodies.
So your anti CCP anibodies are very specific for rhumatoid. Right, it's usually
over ninety percent specific to the disease. So if they ask for the most
specific test for RA, generally this is going to be your anti CCP,
whereas rheumatoid factor is more sensitive but not as specific. And as I mentioned
before, if you can remember which is the specific antibody for RA. Specific
is spelled with two c's, look for the antibody that has two season it
that's your anti CCP. Question five. Last one to twenty six year old
female presents to the office complaining of fatigue, joint pain, and a low
grade fever for the past few weeks. She also reports that she develops a
painful rash after being in the sun just for a short period of time.
Physical exam, you notice a rash that is distributed over the cheeks and nose,
sparing the nasal labial folds, as well as diffuse discoid lesions. What
would be the best initial test to order in this patient? So that is
going to be your anti nuclear antibody, your ANA. So this patient has
systemic lupus or athematosis or obviously this should be at the top of your list
of differentials. Anytime you have a young female of childbearing age, complaining of
joint pain, rash and fever, always be considering lupus. On exam,
she has the classic melar butterfly rash that spares the naso labial folds as well
as the discoid lesions, and she describes a photosensitive rash that burn after a
short period of time in the sun. So the best initial or screening test
in a patient you suspect may have lupus is going to be your ANA anti
nuclear antibody. So it's not a specific test, but very sensitive, and
this is where you'll always start when screening for lupus. Then after obviously you
proceed to your more specific antibodies, your anti double stranded DNA and your anti
Smith antibodies. All right, So that was Rheumatology Part two. I hope
that was helpful. Thank you as always for listening to the podcast, and
good luck on your pants, your pantor your ears, and good luck in
PA school.