Hello, and welcome to systemic lupus erythematosus.
Let’s take a look at SLE.
SLE is a chronic autoimmune syndrome
characterized by multisystem involvement.
Whenever you have immune complexes
that are being involved, think of SLE as being an autoantibody disease
with tons of immune complexes.
Whenever you have immune complex being activated,
then you’ll have a depletion of your complement factors.
That’s a philosophy that we use in medicine basic with any pathology.
For example, if there’s TTP, or ITP, or HUS,
then the thrombi are then going to consume your platelets.
If it’s a DIC, not only will it consume your platelet
but then also, decrease your coag factors, correct?
The point is here, you’re gonna activate your complement
and therefore, you’ll have low serum C3, C4.
In fact, all complement factors are going to be depleted.
This is then represented by a component called CH50.
So if you take all your complement factors including your MAC complex and such,
you’re gonna find these to be decreased.
The exact causality is unclear
but we do know that the T and B cells are involved and SLE
is the main disease or mainly seen in patients
who are women and of African-American descent.
The clinical presentation can be mild to severe.
The cardinal signs however include the following.
Take a look at the young child here.
You take a look at the cheeks and it looks like there is a butterfly type of rash.
This butterfly type of rash pattern could be a cardinal sign of malar or a cardinal sign
that we call malar rash and please notice that it does spare the nasolabial folds.
So, right here.
The nasolabial folds will then be spared in the rash of SLE of malar pattern.
Oral ulcers and nasopharyngeal ulcers could be associated.
You could have a rash maybe perhaps down the fingers on the extensive surface
that may look like a discoid, I’m going to call it discoid rash.
We have two rash patterns so far.
A butterfly rash known as malar and we have a discoid rash.
There could be photosensitivity, malaise,
arthritis could be involved or even arthralgia.
Pleurisy could be involved.
The systems that could be involved with SLE, the skin, obviously.
We talked about the two patterns, the malar
and the discoid photosensitivity.
There could be oral or nasopharyngeal type of ulcers.
The joints, you might find arthritis.
And the serosa, you could have issues with pleuritis,
pericarditis, or abdominal pain.
You could have a type of mixed nephritic and nephrotic type of issue
such as diffuse proliferative glomerulonephritis.
We talked about those markers and we had discussed ANA
and then the speciating of your ANA
into something called double stranded DNA.
In the CNS, really ultimately, it could be anything, anything.
But it could be seizure psychosis, it could be strokes, it could be edema.
In the heme aspect, you could have autoimmune Coombs test positive type of anemia.
You could have these autoantibodies and then form ITP,
Immune Thrombocytopenic Purpura may result in overall WBC count or lymphopenia.
In terms of blood vessels, you could have coronary issues, maybe the GI.
In the lung, you could have pneumonitis and in the heart.
Here you could have - well, just like rheumatoid arthritis
in which you may have coronary arterial disease.
With SLE, you may also develop CAD
and also have myocarditis as a complication.
The diagnosis would then require 11 criteria.
If four of the 11, whereas is rheumatoid arthritis
we talked about four of the seven, and here in SLE,
four of the 11 if they're to be found to be positive,
then we have a sensitivity and specificity as close to 100% at 96.
That butterfly rash that I showed you earlier,
malar, discoid rash, photosensitivity,
oral or nasopharyngeal ulcers, non-erosive arthritis,
for serositis include your pericarditis and pleuritis,
proteinuria could be as high as 500 milligrams per day,
seizures or psychosis but just about anything in CNS,
and we talked about how there might be autoimmune type of hemolytic anemias,
leukopenia or lymphopenia or thrombocytopenia.
You need four of the 11 and every positive
but that is non-specific anti-unclear antibodies
and when you do find anti-double stranded DNA,
then you should note that you have the kidneys involved
and when you do, you’ll have a mixture of both nephritic and nephrotic syndrome
and commonly, you may then find diffuse proliferative glomerulonephritis
with subendothelial deposits.
How do you manage this?
With photosensitivity and issues with rash, avoid exposure.
You also topical steroids for skin disease.
IV or oral steroids for acute flares.
NSAIDs, importantly, hydroxychloroquine for the arthritis.
That’s important, I repeat once again, hydroxychloroquine for arthritis.
For cytotoxic immunosuppressants such as cyclophosphamide for lupus nephritis.
Always be careful with cyclophosphamide obviously,
worry about hemorrhagic cystitis and such, you look for the savior being Mesna.
Or you have mycophenolate or mofetil.
Rituximab is a CD21 inhibitor, in other words,
it’s a B cell-depleting agent, keep that in mind.
Management for SLE.