About
Scleroderma
Scleroderma, or systemic sclerosis, is
a chronic connective tissue disease generally classified as
one of the autoimmune rheumatic diseases.
The word “scleroderma” comes
from two Greek words: “sclero” meaning hard, and
“derma” meaning skin. Hardening of the skin is
one of the most visible manifestations of the disease. The
disease has been called “progressive systemic sclerosis,”
but the use of that term has been discouraged since it has
been found that scleroderma is not necessarily progressive.
The disease may take several forms which will be explained
later. There is also much variability among patients.
What scleroderma is not
Scleroderma is not contagious, it is not
infectious, it is not cancerous or malignant.
How serious
is scleroderma?
Any chronic disease can be serious. The
symptoms of scleroderma vary greatly from individual to individual,
and the effects of scleroderma can range from very mild to
life-threatening. The seriousness will depend on what parts
of the body are affected and the extent to which they are
affected. A mild case can become more serious if not properly
treated. Prompt and proper diagnosis and treatment by qualified
physicians may minimize the symptoms of scleroderma and lessen
the chance for irreversible damage.
How is scleroderma diagnosed?
The diagnostic process may require consultation with rheumatologists
(arthritis specialists), and/or dermatologists (skin specialists)
and require blood studies and numerous other specialized tests
depending upon which organs are affected.
Who develops
scleroderma, and when?
There are an estimated 300,000 people who
have scleroderma, of which 80,000 to 100,000 people in the
United States have the systemic form of the disease. If those
with Raynaud's Phenomenon and one symptom of scleroderma were
counted, the figure would be closer to 150,000. Also, there
are many more people with the localized form of the disease.
International incidence is unknown, but it has been reported
worldwide. Since scleroderma presents with symptoms similar
to other autoimmune diseases, diagnosis is difficult and there
may be many misdiagnosed or undiagnosed cases as well.
Localized scleroderma is more common in children, whereas
systemic scleroderma is more common in adults. Overall female
patients outnumber male patients about four to one, and the
average age at diagnosis is in the forties.
Factors other than sex, such as race and ethnic background,
may influence the risk of getting scleroderma, the age of
onset, and the pattern or severity of internal organ involvement.
The reasons for this are not clear. Although scleroderma is
not directly inherited, some scientists feel there is a slight
predisposition to it in families with a history of rheumatic
diseases.
However, scleroderma can develop and is found in every age
group from infants to the elderly, but its onset is most frequent
between the ages of 25 to 55. When doctors say "usually"
or "for the most part," the reader should understand
that variations frequently occur. Many patients get alarmed
when they read medical information that seems to contradict
their own experiences, and conclude that what has happened
to them is not supposed to happen. There are many exceptions
to the rules in scleroderma, perhaps more so than in other
diseases. Each case is different, and information should be
discussed with your own doctor.
What causes
scleroderma?
The exact cause or causes of scleroderma
are still unknown, but scientists and medical investigators
in a wide variety of fields are working hard to make those
determinations. It is known that scleroderma involves overproduction
of collagen.
Is scleroderma
genetic?
Most patients do not have any relatives with scleroderma
and their children do not get scleroderma. Research indicates
that there is a susceptibility gene which raises the likelihood
of getting scleroderma, but by itself does not cause the disease.
What is the treatment for
scleroderma?
At the present time, there is no cure for scleroderma, but
there are many treatments available. Some are directed at
particular symptoms like heartburn, which can be controlled
by medications called proton pump inhibitors or medicine to
improve the motion of the bowel. Some treatments are directed
at decreasing the activity of the immune system. Some people
with mild disease may not need medication at all and occasionally
people can go off treatment when their scleroderma is no longer
active. Because there is so much variation from one person
to another there is great variation in the treatments prescribed.
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