Treatment of Scleroderma
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Because scleroderma can affect many different organs and
organ systems, you may have several different doctors involved in your
care. Typically, care will be managed by a rheumatologist, a specialist
who treats people with diseases of the joints, bones, muscles, and
immune system. Your rheumatologist may refer you to other specialists,
depending on the specific problems you are having: for example, a
dermatologist for the treatment of skin symptoms, a nephrologist for
kidney complications, a cardiologist for heart complications, a
gastroenterologist for problems of the digestive tract, and a pulmonary
specialist for lung involvement.
In addition to doctors, professionals like nurse
practitioners, physician assistants, physical or occupational
therapists, psychologists, and social workers may play a role in your
care. Dentists, orthodontists, and even speech therapists can treat oral
complications that arise from thickening of tissues in and around the
mouth and on the face.
Currently, there is no treatment that controls or stops
the underlying problem--the overproduction of collagen--in all forms of
scleroderma. Thus, treatment and management focus on relieving symptoms
and limiting damage. Your treatment will depend on the particular
problems you are having. Some treatments will be prescribed or given by
your physician. Others are things you can do on your own.
Here are some of the potential problems that can occur in
systemic scleroderma and the medical and nonmedical treatments for them.
(These problems do not occur as a result or complication of localized
[Note: This is not a complete listing of problems or
their treatments. Different people experience different problems with
scleroderma and not all treatments work equally well for all people.
Work with your doctor to find the best treatment for your specific
Raynaud's phenomenon: One of the most common
problems associated with scleroderma, Raynaud's phenomenon can be
uncomfortable and can lead to painful skin ulcers on the fingertips.
Smoking makes the condition worse. The following measures may make you
more comfortable and help prevent problems:
- Don't smoke! Smoking narrows the blood vessels even more and makes
Raynaud's phenomenon worse.
- Dress warmly, with special attention to hands and feet. Dress in
layers and try to stay indoors during cold weather.
- Use biofeedback (to control various body processes that are not
normally thought of as being under conscious control) and relaxation
- For severe cases, speak to your doctor about prescribing drugs
called calcium channel blockers, such as nifedipine (Procardia), which
can open up small blood vessels and improve circulation. Other drugs
are in development and may become available in the future.
- If Raynaud's leads to skin sores or ulcers, increasing your dose
of calcium channel blockers (under the direction of your doctor ONLY)
may help. You can also protect skin ulcers from further injury or
infection by applying nitroglycerine paste or antibiotic cream. Severe
ulcerations on the fingertips can be treated with bioengineered
More than 70 percent of people with scleroderma first notice this
problem when their fingers turn cold or blue, typically in response to
cold temperatures or emotional distress. Raynaud's phenomenon, as the
condition is called, may precede scleroderma by years. In many people,
however, Raynaud's phenomenon is unrelated to scleroderma, but may
signal damage to the blood vessels supplying the hands arising from
such conditions as occupational injuries (from using jackhammers, for
example), trauma, excessive smoking, circulatory problems, and drug
use or exposure to toxic substances. For some people, cold fingers
(and toes) are the extent of the problem and are little more than a
nuisance. For others, the condition can worsen and lead to puffy
fingers, finger ulcers, and other complications that require
Stiff, painful joints: In diffuse systemic
sclerosis, hand joints can stiffen because of hardened skin around the
joints or inflammation of the joints themselves. Other joints can also
become stiff and swollen. The following may help:
- Exercise regularly. Ask your doctor or physical therapist about an
exercise plan that will help you increase and maintain range of motion
in affected joints. Swimming can help maintain muscle strength,
flexibility, and joint mobility.
- Use acetaminophen or an over-the-counter or prescription
nonsteroidal anti-inflammatory drug, as recommended by your doctor, to
help relieve joint or muscle pain. If pain is severe, speak to a
rheumatologist about the possibility of prescription-strength drugs to
ease pain and inflammation.
- Learn to do things in a new way. A physical or occupational
therapist can help you learn to perform daily tasks, such as lifting
and carrying objects or opening doors, in ways that will put less
stress on tender joints.
Skin problems: When too much collagen builds up in
the skin, it crowds out sweat and oil glands, causing the skin to become
dry and stiff. If your skin is affected, you may need to see a
dermatologist. To ease dry skin, try the following:
- Apply oil-based creams and lotions frequently, and always right
- Apply sunscreen before you venture outdoors, to protect against
further damage by the sun's rays.
- Use humidifiers to moisten the air in your home in colder winter
climates. (Clean humidifiers often to stop bacteria from growing in
- Avoid very hot baths and showers, as hot water dries the
- Avoid harsh soaps, household cleaners, and caustic chemicals, if
at all possible. If that's not possible, be sure to wear rubber gloves
when you use such products.
- Exercise regularly. Exercise, especially swimming, stimulates
blood circulation to affected areas.
Dry mouth and dental problems: Dental problems are
common in people with scleroderma for a number of reasons: tightening
facial skin can make the mouth opening smaller and narrower, which makes
it hard to care for teeth; dry mouth due to salivary gland damage speeds
up tooth decay; and damage to connective tissues in the mouth can lead
to loose teeth. You can avoid tooth and gum problems in several
- Brush and floss your teeth regularly. (If hand pain and stiffness
make this difficult, consult your doctor or an occupational therapist
about specially made toothbrush handles and devices to make flossing
- Have regular dental checkups. Contact your dentist immediately if
you experience mouth sores, mouth pain, or loose teeth.
- If decay is a problem, ask your dentist about fluoride rinses or
prescription toothpastes that remineralize and harden tooth
- Consult a physical therapist about facial exercises to help keep
your mouth and face more flexible.
- Keep your mouth moist by drinking plenty of water, sucking ice
chips, using sugarless gum and hard candy, and avoiding mouthwashes
with alcohol. If dry mouth still bothers you, ask your doctor about a
saliva substitute or a prescription medication called pilocarpine
hydrochloride (Salagen) that can stimulate the flow of saliva.
Gastrointestinal (GI) problems: Systemic sclerosis
can affect any part of the digestive system. As a result, you may
experience problems such as heartburn, difficulty swallowing, early
satiety (the feeling of being full after you've barely started eating),
or intestinal complaints such as diarrhea, constipation, and gas. In
cases where the intestines are damaged, your body may have difficulty
absorbing nutrients from food. Although GI problems are diverse, here
are some things that might help at least some of the problems you
- Eat small, frequent meals.
- Raise the head of your bed with blocks, and stand or sit for at
least an hour (preferably two or three) after eating to keep stomach
contents from backing up into the esophagus.
- Avoid late-night meals, spicy or fatty foods, and alcohol and
caffeine, which can aggravate GI distress.
- Chew foods well and eat moist, soft foods. If you have difficulty
swallowing or if your body doesn't absorb nutrients properly, your
doctor may prescribe a special diet.
- Ask your doctor about prescription medications for problems such
as diarrhea, constipation, and heartburn. Some drugs called proton
pump inhibitors are highly effective against heartburn. Oral
antibiotics may stop bacterial overgrowth in the bowel that can be a
cause of diarrhea in some people with systemic sclerosis.
Lung damage: About 10 to 15 percent of people with
systemic sclerosis develop severe lung disease, which comes in two
forms: pulmonary fibrosis (hardening or scarring of lung tissue because
of excess collagen) and pulmonary hypertension (high blood pressure in
the artery that carries blood from the heart to the lungs). Treatment
for the two conditions is different.
- Pulmonary fibrosis may be treated with drugs that suppress the
immune system such as cyclophosphamide (Cytoxan) or azathioprine
(Imuran), along with low doses of corticosteroids.
- Pulmonary hypertension may be treated with drugs that dilate the
blood vessels such as prostacyclin (Iloprost).
Regardless of the problem or its treatment, your role in
the treatment process is essentially the same. To minimize lung
complications, work closely with your medical team. Do the
- Watch for signs of lung disease, including fatigue, shortness of
breath or difficulty breathing, and swollen feet. Report these
symptoms to your doctor.
- Have your lungs closely checked, using standard lung-function
tests, during the early stages of skin thickening. These tests, which
can find problems at the earliest and most treatable stages, are
needed because lung damage can occur even before you notice any
- Get regular flu and pneumonia vaccines as recommended by your
doctor. Contracting either illness could be dangerous for a person
with lung disease.
Heart problems: About 15 to 20 percent of people
with systemic sclerosis develop heart problems, including scarring and
weakening of the heart (cardiomyopathy), inflamed heart muscle
(myocarditis), and abnormal heart beat (arrhythmia). All of these
problems can be treated. Treatment ranges from drugs to surgery, and
varies depending on the nature of the condition.
Kidney problems: About 15 to 20 percent of people
with diffuse systemic sclerosis develop severe kidney problems,
including loss of kidney function. Because uncontrolled high blood
pressure can quickly lead to kidney failure, it's important that you
take measures to minimize the problem. Things you can do:
- Check your blood pressure regularly and, if you find it to be
high, call your doctor right away.
- If you have kidney problems, take your prescribed medications
faithfully. In the past two decades, drugs known as ACE
(angiotensin-converting enzyme) inhibitors, including captopril
(Capoten), enalapril (Vasotec), and quinapril (Accupril), have made
scleroderma-related kidney failure a less-threatening problem than it
was in the past. But for these drugs to work, you must take
Cosmetic problems: Even if scleroderma doesn't
cause any lasting physical disability, its effects on the skin's
appearance--particularly on the face--can take their toll on your
self-esteem. Fortunately, there are procedures to correct some of the
cosmetic problems scleroderma causes.
- The appearance of telangiectasias, small red spots on the hands
and face caused by swelling of tiny blood vessels beneath the skin,
may be lessened or even eliminated with the use of guided lasers.
- Facial changes of localized scleroderma, such as the en coup de
sabre that may run down the forehead in people with linear
scleroderma, may be corrected through cosmetic surgery. (However, such
surgery is not appropriate for areas of the skin where the disease is