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
Here is a listing 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 scleroderma.
This listing is not complete because 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
More than 90
percent of people with scleroderma have this condition, in which the fingers
and sometimes other extremities change color in response to cold temperature
or anxiety. For many, Raynaud’s phenomenon precedes other manifestations of
the disease. In other people, however, Raynaud’s phenomenon is unrelated to
scleroderma, but may signal damage to the blood vessels supplying the hands
arising from 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 aggressive treatment.
If you have Raynaud’s phenomenon, 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, which governs 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,
which can open up small blood vessels and improve circulation. Other drugs
are in development and may become available.
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 skin.
Stiff, painful joints
systemic sclerosis, hand joints can stiffen due to hardened skin around the
joints or inflammation within them. Other joints can also become stiff and
Stretching exercises under the direction of a physical and/or
occupational therapist are extremely important to prevent loss of joint
motion. These should be started as soon as the diagnosis of scleroderma is
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
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
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 after
Apply sunscreen before you venture outdoors, to protect against further
damage from 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 the
Avoid very hot baths and showers, as hot water dries the skin.
Avoid harsh soaps, household cleaners, and caustic chemicals, if at all
possible. Otherwise, be sure to wear rubber gloves when you use such
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 ways:
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 easier.
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 enamel.
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 prescription medications such as Salagen or Evoxac, 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 have:
Eat small, frequent meals.
To keep stomach contents from backing up into the esophagus, stand or
sit for at least an hour (preferably two or three) after eating. When it
is time to sleep, keep the head of your bed raised using blocks.
Avoid late-night meals, spicy or fatty foods, alcohol, and caffeine,
which can aggravate GI distress.
Eat moist, soft foods, and chew them well. 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.
Virtually all people
with systemic sclerosis have some loss of lung function. Some 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), or with newer medications that
are prescribed specifically for treating pulmonary hypertension.
Regardless of your particular lung problem or its medical
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
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 symptoms.
Get regular flu and pneumonia vaccines as recommended by your doctor.
Contracting either illness could be dangerous for a person with lung
Common among people
with scleroderma, heart problems include 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.
Renal crisis occurs
in about 10 percent of all patients with scleroderma, primarily those with
early diffuse scleroderma. Renal crisis results in severe uncontrolled high
blood pressure, which can quickly lead to kidney failure. It’s very
important that you take measures to identify and treat the hypertension as
soon as it occurs. These are things you can do:
Check your blood pressure regularly. You should also check it if you
have any new or different symptoms such as a headache or shortness of
breath. If the blood pressure is higher than usual, call your doctor right
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), or
lisinopril, have made scleroderma-related kidney failure a less
threatening problem than it used to be. But for these drugs to work, you
must take them as soon as the hypertension is present.
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
reduced 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
National Institute of Arthritis and
Musculoskeletal and Skin Diseases, USA.