Lupus Association of
Tasmania
SCLERODERMA
Defining
Scleroderma
Scleroderma
is a chronic disease of connective tissues (skin, ligaments, cartilage and
bone), which results from an overproduction of the protein collagen. The terms
"sclero" or "sclerosis" refer to the process of hardening, particularly due to
excessive growth of fibrous tissue. "Derma" refers to the skin. Scleroderma
causes a hardening of the skin and other tissues or organs. Scleroderma also can
affect internal organs because connective tissue is found throughout the body.
The
disease can range from mild to life-threatening. When the skin is affected,
symptoms are visible. When internal organs and structures are affected but the
skin is not, the disease may not be apparent. Involvement of internal organs and
tissues signals a more severe form of scleroderma.
Causes
& Risks
The cause of scleroderma is not known. It is considered to be an "autoimmune
disease," which means that the body's immune system reacts against itself,
causing an overproduction of collagen. Some people appear to have a genetic
predisposition to developing the disease, but it is not directly inherited.
Environment also may play a causal role. Despite the body's uncontrolled
production of collagen, scleroderma is not
malignant (cancerous).
Symptoms
of Scleroderma
Symptoms
vary between individuals, but may include:
- Thickening
of the skin
- Swelling
of the hands and feet
- Pain
and stiffness in the joints
- Aching
of the muscles, joints and bones
- Problems
with the digestive system
- Sjogren's
Syndrome (dry mucus membranes)
- Oral,
facial and dental problems
- Complications
of the heart, lungs, kidneys and other internal organs.
- High
blood pressure
- General
weakness and fatigue
- Weight
loss.
There
are several classifications of scleroderma, based on the areas affected and the
degree of severity.
1. Localized (or
"limited") scleroderma:
- Usually
confined to a few areas of skin, such as the fingers or face
- Progresses
slowly, with good prognosis
- Rarely
advances to the systemic form of the disease
- More
common in children.
2.
Systemic (or "diffused") sclerosis:
- Internal
organs and tissues are affected (esophagus, gastrointestinal tract, lungs,
heart, kidneys)
- Blood
vessels, muscles and joints may be affected
- Thickening
of skin on the arms, legs, face, abdomen and back
- May
begin with tissue inflammation, but progresses quickly to hardening
- Can
be degenerative and life-threatening as hardening from scar tissue limits the
function of internal organs.
3.
CREST, a milder form of systemic sclerosis.
People
may exhibit some or all of these "CREST" symptoms:
- Calcinosis:
painful calcium deposits in the skin, appearing as white spots, often on
elbows, knees and fingers
- Raynaud's
phenomenon: spasm of tiny arteries in response to cold or stress. Hinders
blood flow to the finger, toes, ears and tongue.
- Oesophageal
dysfunction: difficulty swallowing because of scarring in the oesophagus. This
may cause acid reflux and heartburn
- Sclerodactyly:
thickening and tightening of skin on the fingers and toes. This can limit
motion
- Telangiectasis:
tiny redspots on the hands, palms, forearems, face and lips. These are caused
by dilated capillaries
Diagnosis
Diagnosis
can be difficult, particularly in the early stage of the disease because many of
the symptoms of scleroderma are common to other connective tissue diseases such
as rheumatoid arthritis or lupus (SLE). The diagnostic process begins with a
comprehensive physical examination. In addition, it may
include:
- Blood
tests to identify various antibodies [antinuclear antibodies (ANAs),
anti-centromere antibody, or anti-Scl70 antibody]
- Nail-fold
capillary test, which takes a skin sample from beneath the fingernail and
studies it under the microscope. The test can detect abnormal capillary
function, which is an early sign of scleroderma
- Various
tests to identify any internal disease (chest X-ray, upper and lower GI
endoscopy, electrocardiogram).
Treatment
Although scleroderma cannot be cured, many of its symptoms can be managed in
most cases. Early treatment is critical, particularly during the first three
years after skin symptoms appear. Organ inflammation and scarring usually occur
within this critical three-year period. After that time, the risk of organ
involvement lessens.
Treatment
focuses on the most troublesome symptoms. It may include:
- Medications
to reduce inflammations
- Lotions
and creams to soften and moisturize skin, including over-the-counter and
prescribed moisturizers
- Medications
to control high blood pressure and prevent kidney failure. · Treatment of
Raynaud's symptoms
- Antacids
for oesophageal heartburn
- Immunosuppressant
drugs to suppress the body's overactive immune system
- Localized
laser therapy to remove telangiectasis (red spots on skin).
A
rheumatologist is usually the primary physician treating scleroderma, but a
dermatologist may also need to be involved to address skin-related symptoms.
Scleroderma
& Quality of Life
Mild and moderate cases of scleroderma do not interfere substantially with
quality of life. People with the more aggressive, systemic form of scleroderma
may be limited as scarring of internal organs causes loss of functioning.
Advanced systemic sclerosis can be life-threatening, so medical monitoring is
essential.
Talk it over with your GP and join your local LUPUS support group to meet with and talk to other scleroderma sufferers.
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