Softening the Blow of Scleroderma

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Hormone looks promising for connective tissue disorder

By Jeff Kelliher
HealthScout Reporter

TUESDAY, June 6 (HealthScout) -- A hormone
related to pregnancy may be a relief to the
estimated 300,000 Americans with scleroderma, a
disease that causes hardening and thickening of the
skin and internal organs.

A study of 68 patients with moderate to severe
scleroderma found significant improvement among
those who Stook low doses of the hormone,
recombinant human relaxin, during a six-month trial.
Scleroderma is a painful condition that until now
has had no direct treatment. It involves
over-production of collagen, the main protein found
in scar tissue. About 80 percent of the people with
the disease are women in their childbearing years.
Scleroderma leads to skin changes that can alter a
person's facial features and cause organ systems
including the lungs, heart, gastrointestinal tract and
kidneys to fail.

The study was done at 13 medical centers across
the country on patients between the ages of 18 and
70. It was funded by Connetics Corp. of Palo
Alto, Calif., which makes relaxin.

Using a 50-point scale that measures skin
thickness, researchers determined that none of the
patients on relaxin got worse. In addition, 71
percent improved their scores by at least seven
points and 52 percent improved by at least 10
points.

The findings appear in the June 6 issue of the
Annals of Internal Medicine.
"Relaxin in pregnancy loosens the ligaments of the
birth canal in preparation for labor," says Dr. James
Seibold, director of the scleroderma program at the
University of Medicine and Dentistry of New
Jersey-Robert Wood Johnson Medical School.
"Those ligaments are mainly made of collagen and
since collagen is the principal problem faced by
people with scleroderma, investigation of relaxin's
effects on the biology of scarring was begun."

Seibold, the study's lead author, says relaxin
appears to fight scleroderma in three ways: it
suppresses production of new collagen; it promotes
production of collagenase, an enzyme that breaks
down collagen; and it reduces production of a
compound that inhibits collagenase.

"Everything that got better had to do with a
measure of scarring," says Seibold. "The [use of
relaxin] is biologically plausible and the results of
the study are remarkably cohesive."

Less is more?

Dr. Frank Arnett, a scleroderma expert from
Houston, says he's encouraged by Seibold's work
with relaxin. But he also has a few reservations.

"Relaxin works in low doses but not in high doses,
which gives me some reservations about it," says
Arnett. "It doesn't make sense. I'm concerned
about whether the low dose results are due to
chance alone or due to the drug itself."

While he admits that no other drug has been shown
to be effective against the disease, Arnett still hopes
more can be done.

"Relaxin is not the final answer," says Arnett. "We
certainly need better drugs than this, but it's a good
first start."
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Peter Giusti, executive director of the Scleroderma
Foundation, says he's excited to learn of advances
in treatments for the disorder.

"Treatment of scleroderma is difficult -- challenging
at best," says Giusti, whose foundation funds $1.5
million in scleroderma research each year. "This
particular study is promising and we're hopeful
relaxin will be successful in helping our members."

What To Do

Scleroderma can hamper even the simplest of daily
tasks, such as getting dressed or turning a key. At
its worst it can interfere with the ability to swallow
and digest food, to catch a breath during physical
activity, and to control high blood pressure.

Symptoms can vary depending on the organ
systems affected. These tend to include the lungs,
heart, gut, kidneys and musculoskeletal system.
About half of the people with scleroderma live for
only 10 years following diagnosis. Between 90
percent to 95 percent of people with scleroderma
also have Raynaud's syndrome, an abnormal
sensitivity to cold in the hands and feet.

June is National Scleroderma Awareness Month.
This year's spokeswoman for the Scleroderma
Foundation is Tylyn John, a former Playboy
Playmate (Miss March, 1992) who was diagnosed
with the disease in 1999 a year after the birth of her
son.

To learn more about scleroderma, you can visit the
Scleroderma Foundation. You can also go to
Scleroderma from A to Z.