cleroderma,
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
in the United States who have scleroderma, about one
third of whom have the systemic form of scleroderma.
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. |