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- So the treatment of scleroderma-associated
myopathy is still unknown,
and the reason is because
we just still don't know enough about the disease.
But certainly, as I mentioned before,
if someone has true overlap myositis,
like polymyositis or dermatomyositis,
then we would treat with immunosuppression,
such as prednisone and other steroid-sparing agents,
such as mycophenolate or methotrexate
or even IVIG if someone has severe weakness.
But, again, we don't know what is the best way
for people who have scleroderma and muscle disease together.
Although we don't know how best to treat on occasion,
the workup, which includes potentially a biopsy,
can help us determine if it is truly overlap myositis.
Or if it's a fibrosing myopathy,
it would be a little bit of a different approach
in terms of treatment.
But, again, it's really dependent
on how the patient is doing clinically
and what their clinical presentation is
to determine the final treatment algorithm for that patient.
So it's gonna be very patient specific.
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In scleroderma-associated muscle disease
and in myositis in general, also I think
the multidisciplinary approach is very important
because if our patients have severe diaphragmatic weakness,
we wanna work with the pulmonologist
to maximize treatment and even consider
pulmonary rehab for these type of patients.
Another person that's very important
in that kind of treatment plan
would be a physical therapist
or PM and R physical medicine rehab
to adopt an exercise program.
And lastly I think when you have
two overlap diseases like scleroderma and myositis,
you wanna really work closely with your rheumatologist
plus other providers who can
formulate a very specific treatment plan
for your disease process.
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So for the treatment of scleroderma-associated
muscle disease, if someone has inflammatory myositis,
we typically use prednisone or immunosuppression.
But high doses of prednisone have been associated
with renal crisis, which is renal failure,
in patients with scleroderma.
And so using high doses of prednisone
can lead to this kind of adverse effect,
including other adverse effects,
but in this specific patient population,
patients who have scleroderma,
it's best not to use high-dose steroids.
So it's very important that the patient
holds hands with the rheumatologist
to make sure that the steroid dose is adequate
but not too high but certainly not too low,
so it's gonna be important to talk
to your doctor about that as well.
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Patients with scleroderma-associated myopathy
can certainly have flares.
Flares can also be seen in myositis and lupus
and all these other autoimmune conditions.
So, if we are on treatment,
certainly if someone's doing well,
we say that they're in remission,
kind of a chemical-induced remission
from the medicines that we put them on.
But if they get flares,
that means they're getting muscle weakness
despite you being on those medications.
So you can get sudden rise in CK,
sudden decrease in muscle endurance,
or weakness that may come up
even after many months of treatment.
But that's a little bit atypical.
Typically once we treat a patient,
I feel like they tend to do pretty well,
but that's why you have to have
close communication with your rheumatologist
so that if you do feel weaker,
we have to figure out is it because this is a flare?
Or is it a progression of your disease?
Because flare just means that you're
feeling a little bit worse now,
and we can treat it with some medications,
and it should go away.
Or is it there's a rapid progression of the disease
that's just not halting despite
all the treatment we're giving you?
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Patients who have scleroderma-associated muscle disease,
it's not a predominantly recognized entity
that scleroderma patients can get muscle disease,
and so I feel like even physicians
potentially can say and attribute your weakness
and muscle fatigue and muscle pain, oh,
this is all just from the autoimmune disease
and just say it's all from the scleroderma.
It's because your skin is getting tight.
But, we see that that's not the case.
Certainly that could potentially happen,
but we shouldn't ignore those symptoms.
If you have those symptoms and you have scleroderma,
it shouldn't be brushed aside as saying
that it's from your scleroderma only
because you have contractures
or because you have a skin tightness.
But it should be heeded, and it should result in,
I believe, a thorough workup for muscle disease.
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