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- The early symptoms of scleroderma often present
with something called Raynaud's Phenomenon.
Raynaud's involves spasm of the blood vessels,
usually in the periphery, meaning in the hands or feet,
during exposure to cold temperatures,
changes in relative temperature,
or exposure to stressful events.
And during a Raynaud's episode, patients will develop
discoloration of their fingers or toes, or both,
and the discoloration is usually initiated
by an onset of pallor,
or white color of the fingers, which is often followed by
a blue discoloration, and finally with a blushing
of the fingers.
So that's one of the most common early signs.
Associated with Raynaud's is something called
a digital ulcer.
It doesn't happen in everybody.
Only about half of patients ever get these ulcers
and scleroderma, but they can be very painful
and they affect the very distal tip of the fingertip.
You can also have little indentions,
like a little pencil poked into the end of the finger,
and they can be painful.
Raynaud's is definitely the most common first sign
of scleroderma, but it can also be associated
with many other autoimmune diseases,
such as thyroid disease, other connective tissue diseases.
It even can be associated with
non-connective tissue diseases,
so it's definitely not specific.
But it is seen in about 93 to 95% of our patients, so it's
common in our population.
Other early signs of scleroderma can include
gastroesophageal reflux disease, although this again is very
common in the general population, and so it's seen in many
patients without scleroderma.
Other early signs can include puffy fingers.
Patients will often not be able to wear their rings
that they've worn for many years before, and this is in
the absence of high sodium intake
or significant weight gain.
Some of the signs and symptoms of diffuse scleroderma,
the biggest distinguishing feature, includes the skin
thickening that can occur on the upper arms,
thighs, chest and abdomen.
This is different from limited scleroderma as those area of
skin are definitely not involved.
And because the skin thickening can affect more
proximal areas of the body, many patients with diffuse
scleroderma often have trouble moving larger joints such
as the shoulders and hips from early on in their disease.
This can cause significant disability.
In addition, many patients with diffuse scleroderma will
also have involvement of muscles and muscle weakness
and muscle disease can be inflammatory
or fibrotic in scleroderma.
This can be a big problem for patients with diffuse disease.
Patients with diffuse disease can also be affected by
interstitial lung disease, or fibrosis of the lungs,
by kidney involvement or scleroderma renal crisis.
And they can also have something called
pulmonary hypertension.
Crest is a subtype of limited scleroderma.
It's spelled just like it's spelled; C-R-E-S-T,
and it's an acronym that stands for
calcinosis, Raynaud's phenomenon, esophageal dysmotility,
sclerodactyly, and telangiectasias.
Now, the calcinosis typically involves an inflammatory
lesion in the skin where you can have the deposition of
calcium, or white fluid or crystals
that can be quite painful, and often occur in the elbow
or knees, sometimes in the hands.
The second one that we just discussed before
was Raynaud's Phenomenon, followed by esophageal dysmotility
which is essentially gastroesophageal reflux disease
involving slowing of the movement of the esophagus.
Slerodactyly is the S in crest and it is describing
skin thickening of the fingers.
And telangiectasias are small, dilated postcapillary venules
that appear as red spots on the skin; most commonly seen
on the face, inner lip, mouth, palms of the hands and chest.
And so these are all features that are
associated with crest.
Now in order to be diagnosed with crest, you generally need
three out of five of those features.
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