Scleroderma is the next topic.
Scleroderma in general.
Here, because you have the viscera being
involved, then we have to refer to
what’s known as diffuse or systemic type
of scleroderma versus your limited.
The limited type would be cutaneous.
Limited would be one in which you
would then find skin involvement.
Limited type would be skin
without any viscera involvement.
The skin type of scleroderma
is involved with CREST.
That’s a different topic, with Calcinosis, Raynaud’s,
Esophageal dysmotility, Sclerodactyly and Telangiectasia.
Here with scleroderma, if you’re
thinking about visceral involvement,
not only should you be thinking about the
esophagus, more importantly you’re
thinking about the diaphragm, and also
the kidney, because here the patient
may actually die of renal failure or
maybe perhaps respiratory failure.
But understand here that there will be diffused fibrosis,
leads to loss of lower esophageal sphincter function.
There’s complete absence of
peristalsis; Esophageal dysmotility.
The symptoms of GERD and
dysphagia are very much present.
It is associated with Raynaud’s.
Manometry shows incompetent lower esophageal sphincter,
absent peristalsis because of well, the
esophagus has now become a lead pipe.
That should not be the case.
And it is hard as a pipe.
Therapy - PPI, anti-reflux measure.
Obviously here, you’re thinking about an autoimmune disease
and with scleroderma… here if it’s diffuse
and viscera involvement,
if you remember correctly, here the marker that you’re going to
be looking for is Scl-70 - Scleroderma-70, and topoisomerase.