Obviously scleroderma itself, the first
description that should come to mind is
reflexively, Oh, Fibrosis.
Where is the fibrosis
that you might find?
Well, here there might
be two manifestations
And by that mean there might
be two method by which
scleroderma is developing
in your patient.
It might be the cutaneous type.
And if it's the cutaneous type of
manifestation, then there's going to be
excess deposition of collagen ,
fibrosis on the skin - cutaneous.
Whereas if there's skin and
an organ to be affected,
then that is going to be more
of your systemic type of issue
Is that clear?
It is that black and white
when dealing with scleroderma.
At some point, when you have reviewed
your connective tissue diseases,
and we discuss our sclerodermas,
then we'll take a look at particular
markers that are important for you.
For example, let me show you the
one that you all know so very well
which is CREST, right?
Now if it is esophageal dysmotility
that you find with scleroderma,
read the first statement here,
then you can imagine that your patient
most likely would be suffering from
Well, what if your patient
now has fever,
and also has come back to be
positive for anti-centromere?
that fever and the type of lung issue
that you might find in this patient
would be aspiration pneumonia.
I need you to be very clear
and understand the, well
the pathogenesis of how
the lung is being damaged
This will be
Whereas if there was fibrosis
that's taking place, of the lung
Oh, maybe there wasn't
and so therefore it might
have been nonspecific.
Uh oh, there's quite a bit of
fibrosis taking place in lung
You see "honeycomb appearance"
on your chest x-ray,
Oh my goodness, this is
idiopathic pulmonary fibrosis.
Do you understand?
Just by looking at scleroderma
and you're walking through CREST,
And you're affecting the esophagus,
you have answered choices,
and you missed and all that you've
memorized is scleroderma and fibrosis,
and you're not thinking
because it's a long clinical
vignette and you're tired,
Well there's every possibility
that you missed that your patient
might have been suffering from
esophageal dysmotility from scleroderma
therefore the lung lesion here would be
more of the aspiration type.
Are we clear?
I hope so.
Now, diffuse interstitial disease
or alveolitis seen in patients
with systemic disease more
frequently than those with CREST.
Once again, that is,
that's a huge statement.
Understand the difference.
There are two different patterns
One of these is systemic
disease, what does that mean?
Could the skin be involved?
In addition, what else
is being involved?
Now severe organ.
So in this case, we're talking about
the lung so therefore it will be
diffuse interstitial disease,
so will be quite a bit of fibrosis.
Whereas if it was CREST, you pay
attention to E - esophageal dysmotility
and what's causing lung damage?
Good, aspiration pneumonia.
CREST patients are more likely
to have pulmonary hypertension,
and so therefore look for issues
within the right ventricle
Alright so that's important for you to
pay attention to - the two patterns.