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- The disease activity in scleroderma
can be a challenge to monitor.
We break it down by the organ affected in scleroderma,
because not all patients have every organ involved.
So, in skin, for example, we monitor skin scores over time,
meaning how thick the skin is over time.
And generally, we like to see stability of the skin
or improvement,
once patients start immunosuppressant therapy.
If they continue to progress
and their skin score continues to increase over time,
it suggests that the medicine may not be working
or may need a supplemental medication
to help it fully control the disease.
Patients with lung involvement are monitored over time,
with pulmonary function tests.
Those are tests that measure the volume of air
that is exchanged in the lungs
and the rate at which it happens.
And we monitor pulmonary function tests over time,
usually every four to six months
in patients who are higher-risk
for having progressive interstitial lung disease,
and maybe annually or every other year,
in patients who have had stable lung tests over time
and show no signs of progression.
In patients with muscle involvement,
often times, there are markers in the blood
that can be helpful in monitoring disease activity.
Creatine kinase or CK, and aldolase
are both muscle enzymes that can be detected
with a simple blood draw.
And when patients are not on therapy
and they have disease, those markers are typically high,
either one or both of them.
As patients are treated, we like to see those numbers drop.
However, the most important sign of clinical improvement
in patients is their muscle strength and their function.
And so, the blood tests aren't the only way
to monitor disease activity.
It really depends on the whole picture of the patient
and the physician's assessment.
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Depending on the complication of scleroderma
that is present in a patient,
that will direct the type of therapy that we use.
So patients with significant skin thickening,
inflammatory muscle involvement,
inflammatory cardiac involvement,
progressive interstitial lung disease,
will all be managed with medications that suppress
or modulate the immune system,
whereas patients who really only have some mild Raynaud's,
really limited or no skin thickening,
we don't generally use immunosuppressant medications
for them, because the risks of those medications
will outweigh the benefits.
And in patients who have significant Raynaud's,
we take an alternate route.
And we typically use vasodilators
that improve blood flow to the fingertips and/or toes
and will help profusion so that they're at less of a risk
of having flares of Raynaud's,
they're at less of a risk of developing digital ulcers
and losing tissue from their fingers.
Patients with gastrointestinal dysmotility,
immunosuppression is not the first line for those patients.
We instead use medications that manage symptoms,
such as antacids, lifestyle modification for acid reflux,
dietary modification, empiric antibiotics for patients
with small bacterial overgrowth
and medications to manage constipation.
Therapies vary widely across patients with scleroderma,
depending on the type of scleroderma that someone has.
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