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Derived from the Greek words "sklerosis," meaning
hardness, and "derma," meaning skin, scleroderma literally means hard
skin. Though it is often referred to as if it were a single disease,
scleroderma is really a symptom of a group of diseases that involve the
abnormal growth of connective tissue, which supports the skin and
internal organs. It is sometimes used, therefore, as an umbrella term
for these disorders. In some forms of scleroderma, hard, tight skin is
the extent of this abnormal process. In other forms, however, the
problem goes much deeper, affecting blood vessels and internal organs,
such as the heart, lungs, and kidneys.
Scleroderma is called both a rheumatic (roo-MA-tik)
disease and a connective tissue disease. The term rheumatic disease
refers to a group of conditions characterized by inflammation and/or
pain in the muscles, joints, or fibrous tissue. A connective tissue
disease is one that affects the major substances in the skin, tendons,
In this booklet we'll discuss the forms of scleroderma and
the problems with each of them as well as diagnosis and disease
management. We'll also take a look at what research is telling us about
their possible causes and most effective treatments, and ways to help
people with scleroderma live longer, healthier, and more productive
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The group of diseases we call scleroderma falls into two
main classes: localized scleroderma and systemic sclerosis. (Localized
diseases affect only certain parts of the body; systemic diseases can
affect the whole body.) Both groups include subgroups. (See chart.)
Although there are different ways these groups and subgroups may be
broken down or referred to (and your doctor may use different terms from
what you see here), the following is a common way of classifying these
Localized types of scleroderma are those limited to the
skin and related tissues and, in some cases, the muscle below. Internal
organs are not affected by localized scleroderma, and localized
scleroderma can never progress to the systemic form of the disease.
Often, localized conditions improve or go away on their own over time,
but the skin changes and damage that occur when the disease is active
can be permanent. For some people, localized scleroderma is serious and
There are two generally recognized types of localized
Morphea: Morphea (mor-FEE-ah) comes from a Greek
word that means "form" or "structure." The word refers to local patches
of scleroderma. The first signs of the disease are reddish patches of
skin that thicken into firm, oval-shaped areas. The center of each patch
becomes ivory colored with violet borders. These patches sweat very
little and have little hair growth. Patches appear most often on the
chest, stomach, and back. Sometimes they appear on the face, arms, and
Morphea can be either localized or generalized.
Localized morphea limits itself to one or several patches,
ranging in size from a half-inch to 12 inches in diameter. The condition
sometimes appears on areas treated by radiation therapy. Some people
have both morphea and linear scleroderma (described below). The disease
is referred to as generalized morphea when the skin patches
become very hard and dark and spread over larger areas of the body.
Regardless of the type, morphea generally fades out in 3
to 5 years; however, people are often left with darkened skin patches
and, in rare cases, muscle weakness.
Linear scleroderma: As suggested by its name, the
disease has a single line or band of thickened and/or abnormally colored
skin. Usually, the line runs down an arm or leg, but in some people it
runs down the forehead. People sometimes use the French term en coup
de sabre, or "sword stroke," to describe this highly visible
Systemic Scleroderma (also known as Systemic
Systemic scleroderma, or systemic sclerosis, is the term
for the disease that not only includes the skin, but also involves the
tissues beneath to the blood vessels and major organs. Systemic
sclerosis is typically broken down into diffuse and
limited disease. People with systemic sclerosis often have all or
some of the symptoms that some doctors call CREST, which stands for the
- Calcinosis (KAL-sin-OH-sis): the formation of calcium
deposits in the connective tissues, which can be detected by x ray.
They are typically found on the fingers, hands, face, and trunk and on
the skin above elbows and knees. When the deposits break through the
skin, painful ulcers can result.
- Raynaud's (ray-NOHZ) phenomenon: a condition in
which the small blood vessels of the hands and/or feet contract in
response to cold or anxiety. As the vessels contract, the hands or
feet turn white and cold, then blue. As blood flow returns, they
become red. Fingertip tissues may suffer damage, leading to ulcers,
scars, or gangrene.
- Esophageal (eh-SOFF-uh-GEE-ul) dysfunction: impaired
function of the esophagus (the tube connecting the throat and the
stomach) that occurs when smooth muscles in the esophagus lose normal
movement. In the upper esophagus, the result can be swallowing
difficulties; in the lower esophagus, the problem can cause chronic
heartburn or inflammation.
- Sclerodactyly (SKLER-oh-DAK-till-ee): thick and tight skin
on the fingers, resulting from deposits of excess collagen within skin
layers. The condition makes it harder to bend or straighten the
fingers. The skin may also appear shiny and darkened, with hair
- Telangiectasias (tel-AN-jee-ek-TAY-zee-uhs): small red
spots on the hands and face that are caused by the swelling of tiny
blood vessels. While not painful, these red spots can create cosmetic
Limited scleroderma: Limited scleroderma typically
comes on gradually and affects the skin only in certain areas: the
fingers, hands, face, lower arms, and legs. Many people with limited
disease have Raynaud's phenomenon for years before skin thickening
starts. Others start out with skin problems over much of the body, which
improves over time, leaving only the face and hands with tight,
thickened skin. Telangiectasias and calcinosis often follow. Because of
the predominance of CREST in people with limited disease, some doctors
refer to limited disease as the CREST syndrome.
Diffuse scleroderma: Diffuse scleroderma typically
comes on suddenly. Skin thickening occurs quickly and over much of the
body, affecting the hands, face, upper arms, upper legs, chest, and
stomach in a symmetrical fashion (for example, if one arm or one side of
the trunk is affected, the other is also affected). Some people may have
more area of their skin affected than others. Internally, it can damage
key organs such as the heart, lungs, and kidneys.
People with diffuse disease are often tired, lose appetite
and weight, and have joint swelling and/or pain. Skin changes can cause
the skin to swell, appear shiny, and feel tight and itchy.
The damage of diffuse scleroderma typically occurs over a
few years. After the first 3 to 5 years, people with diffuse disease
often enter a stable phase lasting for varying lengths of time. During
this phase, skin thickness and appearance stay about the same. Damage to
internal organs progresses little, if at all. Symptoms also subside:
joint pain eases, fatigue lessens, and appetite returns.
Gradually, however, the skin starts to change again. Less
collagen is made and the body seems to get rid of the excess collagen.
This process, called "softening," tends to occur in reverse order of the
thickening process: the last areas thickened are the first to begin
softening. Some patients' skin returns to a somewhat normal state, while
other patients are left with thin, fragile skin without hair or sweat
glands. More serious damage to heart, lungs, or kidneys is unlikely to
occur unless previous damage leads to more advanced deterioration.
People with diffuse scleroderma face the most serious
long-term outlook if they develop severe kidney, lung, digestive, or
heart problems. Fortunately, less than one-third of patients with
diffuse disease develop these problems. Early diagnosis and continual
and careful monitoring are important.
Sine scleroderma: Some doctors break systemic
sclerosis down into a third subset called systemic sclerosis sine
(SEEN-ay, Latin for "without") scleroderma. Sine may resemble either
limited or diffuse systemic sclerosis, causing changes in the lungs,
kidneys, and blood vessels. However, there is one key difference between
sine and other forms of systemic sclerosis: it does not affect the
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Although scientists don't know exactly what causes
scleroderma, they are certain that people cannot catch it from or
transmit it to others. Studies of twins suggest it is also not
inherited. Scientists suspect that scleroderma comes from several
factors that may include:
Abnormal immune or inflammatory activity: Like many
other rheumatic disorders, scleroderma is believed to be an autoimmune
disease. An autoimmune disease is one in which the immune system, for
unknown reasons, turns against one's own body.
In scleroderma, the immune system is thought to stimulate
cells called fibroblasts to produce too much collagen. In scleroderma,
collagen forms thick connective tissue that builds up around the cells
of the skin and internal organs. In milder forms, the effects of this
buildup are limited to the skin and blood vessels. In more serious
forms, it also can interfere with normal functioning of skin, blood
vessels, joints, and internal organs.
Genetic makeup: While genes seem to put certain
people at risk for scleroderma and play a role in its course, the
disease is not passed from parent to child like some genetic
However, some research suggests that having children may
increase a woman's risk of scleroderma. Scientists have learned that
when a woman is pregnant, cells from her baby can pass through the
placenta, enter her blood stream, and linger in her body--in some cases,
for many years after the child's birth. Recently, scientists have found
fetal cells from pregnancies of years past in the skin lesions of some
women with scleroderma. They think that these cells, which are different
from the woman's own cells, may either begin an immune reaction to the
woman's own tissues or trigger a response by the woman's immune system
to rid her body of those cells. Either way, the woman's healthy tissues
may be damaged in the process. Further studies are needed to find out if
fetal cells play a role in the disease.
Environmental triggers: Research suggests that
exposure to some environmental factors may trigger the disease in people
who are genetically predisposed to it. Suspected triggers include viral
infections, certain adhesive and coating materials, and organic solvents
such as vinyl chloride or trichloroethylene. In the past, some people
believed that silicone breast implants might have been a factor in
developing connective tissue diseases such as scleroderma. But several
studies have not shown evidence of a connection.
Hormones: By the middle to late childbearing years
(ages 30 to 55), women develop scleroderma at a rate 7 to 12 times
higher than men. Because of female predominance at this and all ages,
scientists suspect that something distinctly feminine, such as the
hormone estrogen, plays a role in the disease. So far, the role of
estrogen or other female hormones has not been proven.
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Although scleroderma is more common in women, the disease
also occurs in men and children. It affects people of all races and
ethnic groups. However, there are some patterns by disease type. For
- Localized forms of scleroderma are more common in people of
European descent than in African Americans.
- Morphea usually appears between the ages of 20 and 40.
- Linear scleroderma usually occurs in children or teenagers.
- Systemic scleroderma, whether limited or diffuse, typically occurs
in people from 30 to 50 years old. It affects more women of African
American than European descent.
Because scleroderma can be hard to diagnose and it
overlaps with or resembles other diseases, scientists can only estimate
how many cases there actually are. Estimates for the number of people in
the United States with systemic sclerosis range from 40,000 to 165,000.
By contrast, a survey that included all scleroderma-related disorders,
including Raynaud's phenomenon, suggested a number between 250,000 and
For some people, scleroderma (particularly the localized
forms) is fairly mild and resolves with time. But for others, living
with the disease and its effects day to day has a significant impact on
their quality of life.
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Having a chronic disease can affect almost every aspect of
your life, from family relationships to holding a job. For people with
scleroderma, there may be other concerns about appearance or even the
ability to dress, bathe, or handle the most basic daily tasks. Here are
some areas in which scleroderma could intrude.
Appearance and self-esteem: Aside from the initial
concerns about health and longevity, one of the first fears people with
scleroderma have is how the disease will affect their appearance. Thick,
hardened skin can be difficult to accept, particularly on the face.
Systemic scleroderma may result in facial changes that eventually cause
the opening to the mouth to become smaller and the upper lip to
virtually disappear. Linear scleroderma may leave its mark on the
forehead. Although these problems can't always be prevented, their
effects may be minimized with proper treatment and skin care. Special
cosmetics--and in some cases, plastic surgery--can help conceal
Caring for yourself: Tight, hard connective tissue
in the hands can make it difficult to do what were once simple tasks,
such as brushing your teeth and hair, pouring a cup of coffee, using a
knife and fork, unlocking a door, or buttoning a jacket. If you have
trouble using your hands, consult an occupational therapist, who can
recommend new ways of doing things or devices to make tasks easier.
Devices as simple as Velcro* fasteners and built-up brush handles can
help you be more independent.
* Brand names included in this booklet are
provided as examples only, and their inclusion does not mean that
these products are endorsed by the National Institutes of Health or
any other Government agency. Also, if a particular brand name is not
mentioned, this does not mean or imply that the product is
Family relationships: Spouses, children, parents,
and siblings may have trouble understanding why you don't have the
energy to keep house, drive to soccer practice, prepare meals, and hold
a job the way you used to. If your condition isn't that visible, they
may even suggest you are just being lazy. On the other hand, they may be
overly concerned and eager to help you, not allowing you to do the
things you are able to do or giving up their own interests and
activities to be with you. It's important to learn as much about your
form of the disease as you can and share any information you have with
your family. Involving them in counseling or a support group may also
help them better understand the disease and how they can help you.
Sexual relations: Sexual relationships can be
affected when systemic scleroderma enters the picture. For men, the
disease's effects on the blood vessels can lead to problems achieving an
erection. In women, damage to the moisture-producing glands can cause
vaginal dryness that makes intercourse painful. People of either sex may
find they have difficulty moving the way they once did. They may be
self-conscious about their appearance or afraid that their sexual
partner will no longer find them attractive. With communication between
partners, good medical care, and perhaps counseling, many of these
changes can be overcome or at least worked around.
Pregnancy and childbearing: In the past, women with
systemic scleroderma were often advised not to have children. But thanks
to better medical treatments and a better understanding of the disease
itself, that advice is changing. (Pregnancy, for example, is not likely
to be a problem for women with localized scleroderma.) Although blood
vessel involvement in the placenta may cause babies of women with
systemic scleroderma to be born early, many women with the disease can
have safe pregnancies and healthy babies if they follow some
One of the most important pieces of advice is to wait a
few years after the disease starts before attempting a pregnancy. During
the first 3 years you are at the highest risk of developing severe
problems of the heart, lungs, or kidneys that could be harmful to you
and your unborn baby.
If you haven't developed organ problems within 3 years of
the disease's onset, chances are you won't, and pregnancy should be
safe. But it is important to have both your disease and your pregnancy
monitored regularly. You'll probably need to stay in close touch with
the doctor you typically see for your scleroderma as well as an
obstetrician experienced in guiding high-risk pregnancies.
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Depending on your particular symptoms, a diagnosis of
scleroderma may be made by a general internist, a dermatologist (a
doctor who specializes in treating diseases of the skin, hair, and
nails), an orthopaedist (a doctor who treats bone and joint disorders),
a pulmonologist (lung specialist), or a rheumatologist (a doctor
specializing in treatment of rheumatic diseases). A diagnosis of
scleroderma is based largely on the medical history and findings from
the physical exam. To make a diagnosis, your doctor will ask you a lot
of questions about what has happened to you over time and about any
symptoms you may be experiencing. Are you having a problem with
heartburn or swallowing? Are you often tired or achy? Do your hands turn
white in response to anxiety or cold temperatures?
Once your doctor has taken a thorough medical history, he
or she will perform a physical exam. Finding one or more of the
following factors can help the doctor diagnose a certain form of
- Changed skin appearance and texture, including swollen fingers and
hands and tight skin around the hands, face, mouth, or elsewhere.
- Calcium deposits developing under the skin.
- Changes in the tiny blood vessels (capillaries) at the base of the
- Thickened skin patches.
Finally, your doctor may order lab tests to help confirm a
suspected diagnosis. At least two proteins, called antibodies, are
commonly found in the blood of people with scleroderma:
- Antitopoisomerase-1 or Anti-Scl-70 antibodies appear in the blood
of up to 40 percent of people with diffuse systemic sclerosis.
- Anticentromere antibodies are found in the blood of as many as 90
percent of people with limited systemic sclerosis.
A number of other scleroderma-specific antibodies can
occur in people with scleroderma, although less frequently. When
present, however, they are helpful in clinical diagnosis.
Because not all people with scleroderma have these
antibodies and because not all people with the antibodies have
scleroderma, lab test results alone cannot confirm the diagnosis.
In some cases, your doctor may order a skin biopsy (the
surgical removal of a small sample of skin for microscopic examination)
to aid in or help confirm a diagnosis. However, skin biopsies, too, have
their limitations: biopsy results cannot distinguish between localized
and systemic disease, for example.
Diagnosing scleroderma is easiest when a person has
typical symptoms and rapid skin thickening. In other cases, a diagnosis
may take months, or even years, as the disease unfolds and reveals
itself and as the doctor is able to rule out some other potential causes
of the symptoms. In some cases, a diagnosis is never made, because the
symptoms that prompted the visit to the doctor go away on their own.
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Symptoms similar to those seen in scleroderma can occur
with a number of other diseases. Here are some of the most common
- Eosinophilic fasciitis (EF) (EE-oh-SIN-oh-FIL-ik
fa-shi-EYE-tis): a disease that involves the fascia (FA-shuh), the
thin connective tissue around the muscles, particularly those of the
forearms, arms, legs, and trunk. EF causes the muscles to become
encased in collagen, the fibrous protein that makes up tissue such as
the skin and tendons. Permanent shortening of the muscles and tendons,
called contractures, may develop, sometimes causing disfigurement and
problems with joint motion and function. EF may begin after hard
physical exertion. The disease usually fades away after several years,
but people sometimes have relapses. Although the upper layers of the
skin are not thickened in EF, the thickened fascia may cause the skin
to look somewhat like the tight, hard skin of scleroderma. A skin
biopsy easily distinguishes between the two.
- Undifferentiated connective tissue disease (UCTD): a
diagnosis for patients who have some signs and symptoms of various
related diseases, but not enough symptoms of any one disease to make a
definite diagnosis. In other words, their condition hasn't
"differentiated" into a particular connective tissue disease. In time,
UCTD can go in one of three directions: it can change into a systemic
disease such as systemic sclerosis, systemic lupus erythematosus, or
rheumatoid arthritis; it can remain undifferentiated; or it can
- Overlap syndromes: a disease combination in which patients
have symptoms and lab findings characteristic of two or more
At other times, symptoms resembling those of scleroderma
can be the result of an unrelated disease or condition. For example:
- Skin thickening on the fingers and hands also appears with
diabetes, mycosis fungoides, amyloidosis, and adult celiac disease. It
can also result from hand trauma.
- Generalized skin thickening may occur with scleromyxedema,
graft-versus-host disease, porphyria cutanea tarda, and human adjuvant
- Internal organ damage, similar to that seen in systemic
sclerosis, may instead be related to primary pulmonary hypertension,
idiopathic pulmonary fibrosis, or collagenous colitis.
- Raynaud's phenomenon also appears with atherosclerosis or
systemic lupus erythematosus or in the absence of underlying
An explanation of most of these other diseases is beyond the scope
of this booklet. What's important to understand, however, is that scleroderma
isn't always easy to diagnose; it may take time for you and your doctor to
establish a diagnosis. And while having a definite diagnosis may be helpful,
knowing the precise form of your disease is not needed to receive proper treatment.
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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
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Although your doctors direct your treatment, you are the
one who must take your medicine regularly, follow your doctor's advice,
and report any problems promptly. In other words, the relationship
between you and your doctors is a partnership, and you are the most
important partner. Here's what you can do to make the most of this
- Get educated: Knowledge is your best defense against this
disease. Learn as much as you can about scleroderma, both for your own
benefit and to educate the people in your support network (see
- Seek support: Recruit family members, friends, and coworkers to
build a support network. This network will help you get through difficult
times: when you are in pain; when you feel angry, sad, or afraid; when you're
depressed. Also, look for a scleroderma support group in your community
by calling a national scleroderma organization. If you can't find a support
group, you might want to consider organizing one.
- Assemble a health care team: You and your doctors will lead
the team. Other members may include physical and occupational
therapists, a psychologist or social worker, a dentist, and a
- Be patient: Understand that a final diagnosis can be
difficult and may take a long time. Find a doctor with experience
treating people with systemic and localized scleroderma. Then, even if
you don't yet have a diagnosis, you will get understanding and the
right treatment for your symptoms.
- Speak up: When you have problems or notice changes in your
condition, don't feel too self-conscious to speak up during your
appointment or even call your doctor or another member of your health
care team. No problem is too small to inquire about, and early
treatment for any problem can make the disease more manageable for you
and your health care team.
- Don't accept depression: While it's understandable that a
person with a chronic illness like scleroderma would become depressed,
don't accept depression as a normal consequence of your condition. If
depression makes it hard for you to function well, don't hesitate to
ask your health care team for help. You may benefit from speaking with
a psychologist or social worker or from using one of the effective
medications on the market.
- Learn coping skills: Skills like meditation, calming
exercises, and relaxation techniques may help you cope with emotional
difficulties as well as help relieve pain and fatigue. Ask a member of
your health care team to teach you these skills or to refer you to
someone who can.
- Ask the experts: If you have problems doing daily
activities, from brushing your hair and teeth to driving your car,
consult an occupational or physical therapist. They have more helpful
hints and devices than you can probably imagine. Social workers can
often help resolve financial and insurance matters.
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No one can say for sure when--or if--a cure will be found.
But research is providing the next best thing: better ways to treat
symptoms, prevent organ damage, and improve the quality of life for
people with scleroderma. In the past two decades, multidisciplinary
research has also provided new clues to understanding the disease, which
is an important step toward prevention or cure.
Leading the way in funding for this research is the
National Institute of Arthritis and Musculoskeletal and Skin Diseases
(NIAMS), a part of the National Institutes of Health (NIH). Other
sources of funding for scleroderma research include pharmaceutical
companies and organizations such as the Scleroderma Foundation, the
Scleroderma Research Foundation, and the Arthritis Foundation.
Scientists at universities and medical centers throughout the United
States conduct much of this research.
Studies of the immune system, genetics, cell biology, and
molecular biology have helped reveal the causes of scleroderma, improve
existing treatment, and create entirely new treatment approaches.
- The use of a hormone produced in pregnancy to soften skin lesions.
Early studies suggest relaxin, a hormone that helps a woman's body to
stretch to meet the demands of a growing pregnancy and delivery, may
soften the connective tissues of women with scleroderma. The hormone
is believed to work by blocking fibrosis, or the development of
fibrous tissue between the body's cells.
- Finding a gene associated with scleroderma in Oklahoma Choctaw
Native Americans. Scientists believe the gene, which codes for a
protein called fibrillin-1, may put people at risk for the disease.
- The use of the drug Iloprost for pulmonary hypertension. This drug
has increased the quality of life and life expectancy for people with
this dangerous form of lung damage.
- The use of the drug cyclophosphamide (Cytoxan) for lung fibrosis.
One recent study suggested that treating lung problems early with this
immunosuppressive drug may help prevent further damage and increase
chances of survival.
- The increased use of ACE inhibitors for scleroderma-related kidney
problems. For the past two decades, ACE inhibitors have greatly
reduced the risk of kidney failure in people with scleroderma. Now
there is evidence that use of ACE inhibitors can actually heal the
kidneys of people on dialysis for scleroderma-related kidney failure.
As many as half of people who continue ACE inhibitors while on
dialysis may be able to go off dialysis in 12 to 18 months.
- Changes in the tiny blood vessels of people with scleroderma. By
studying these changes, scientists hope to find the cause of cold
sensitivity in Raynaud's phenomenon and how to control the
- Immune system changes (and particularly how those changes affect
the lungs) in people with early diffuse systemic sclerosis.
- The role of blood vessel malfunction, cell death, and autoimmunity
- Skin changes in laboratory mice in which a genetic defect prevents
the breakdown of collagen, leading to thick skin and patchy hair loss.
Scientists hope that by studying these mice, they can answer many
questions about skin changes in scleroderma.
- The effectiveness of various treatments, including (1)
methotrexate, a drug commonly used for rheumatoid arthritis and some
other inflammatory forms of arthritis; (2) collagen peptides
administered orally; (3) halofugione, a drug that inhibits the
synthesis of type I collagen, which is the primary component of
connective tissue; (4) ultraviolet light therapy for localized forms
of scleroderma; and (5) stem cell transfusions, a form of bone marrow
transplant that uses a patient's own cells, for early diffuse systemic
Scleroderma research continues to advance as scientists
and doctors learn more about how the disease develops and its underlying
Recently, the NIAMS funded a Specialized Center of
Research (SCOR) in scleroderma at the University of Texas-Houston. SCOR
scientists are conducting laboratory and clinical research on the
disease. The SCOR approach allows researchers to translate basic science
findings quickly into improved treatment and patient care.
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Scleroderma poses a series of challenges for both patients
and their health care teams. The good news is that scientists, doctors,
and other health care professionals continue to find new answers--ways
to make earlier diagnoses and manage disease better. In addition, active
patient support groups share with, care for, and educate each other. The
impact of all of this activity is that people with scleroderma do much
better and remain active far longer than they did 20 or 30 years ago. As
for tomorrow, patients and the medical community will continue to push
for longer, healthier, and more active lives for people with the
diseases collectively known as scleroderma.
Institute of Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse
College of Rheumatology
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Adult celiac disease--A chronic nutritional
disorder in which the body cannot effectively digest fats and wheat
gluten. The condition, which results in a distended abdomen and loose,
fatty stools, is associated with several autoimmune diseases.
Amyloidosis--A disease in which excessive protein
is deposited around cells in various organs and tissues of the body.
Antibodies--Special proteins produced by the body's
immune system. They recognize and help fight infectious agents, such as
bacteria and other foreign substances that invade the body. The presence
of certain antibodies in the blood can help in making a diagnosis of
some diseases, including some forms of scleroderma.
Atherosclerosis--Abnormal fatty deposits in the
inner layers of large or medium-sized arteries, which can lead to
hardening and narrowing of the arteries and blockages of the blood
supply, especially to the heart.
Autoimmune disease--A disease in which the body's
immune system turns against and damages the body's own tissues.
Calcinosis--The buildup of calcium deposits in the
tissues. It may occur under the skin of the fingers, arms, feet, and
knees, causing pain and infection if the calcium deposits pierce the
surface of the skin.
Calcium channel blockers--Medicines that lower
blood pressure, relieve chest pain, and stabilize normal heart rhythms
by inhibiting calcium movement into the heart muscles and smooth muscle
cells. They are used to treat a variety of conditions and to prevent
circulatory and kidney problems in scleroderma.
Colitis--An inflammatory disease of the large
intestine that results in diarrhea, discharge of mucus and blood,
cramping, and abdominal pain. It is characterized by swelling,
inflammation, and ulceration of the mucous membrane of the
Collagen--A fabric-like material of fibrous threads
that is a key component of the body's connective tissues. In
scleroderma, too much collagen is produced or it is produced in the
wrong places, causing stiff and inflamed skin, blood vessels, and
Connective tissue--Tissues such as skin, tendons,
and cartilage that support and hold body parts together. The chief
component of connective tissue is collagen.
CREST syndrome--An acronym for a collection of
symptoms that occur to some degree in all people with systemic
sclerosis. The symptoms are Calcinosis, Raynaud's
phenomenon, Esophageal dysfunction, Sclerodactyly, and
Telangiectasia. Because of the predominance of CREST symptoms in
people with limited systemic sclerosis, some people use the term CREST
syndrome when referring to that form of the disease.
Eosinophilic fasciitis--A scleroderma-like disorder
(often considered to be a localized form of scleroderma) featuring
inflammation of the fascia (the thin, sheet-like connective tissues
surrounding the muscles and other body structures) and an abnormally
high number of a specific kind of white blood cells (eosinophils). The
result of the inflammation may be fibrous buildup in the skin of arms
and legs, contractures, and carpal tunnel syndrome.
Esophageal dysfunction--Improper functioning of the
esophagus (the tube that attaches the throat to the stomach) that can
lead to heartburn and swallowing problems.
Fibroblast--A type of cell in connective tissue
that secretes proteins, including collagen.
Fibrosis--A condition marked by increased fibrous
tissue that develops between the cells of various organs or tissues. It
is a common feature of scleroderma and some other diseases. Fibrosis
causes hardening or stiffening of tissues in the skin, joints, and
Graft--versus-host disease-A major complication of
bone marrow transplantations and sometimes blood transfusions in which
white blood cells, called lymphocytes, in the marrow or blood attack
tissues in the body into which they were transplanted.
Human adjuvant disease--An autoimmune syndrome in
which the body becomes extremely sensitive to a foreign material
injected into the body.
Mycosis fungoides--A form of lymph cancer
characterized by scaly skin patches. It progresses over several years to
form elevated skin lesions and then tumors.
Pulmonary fibrosis--Hardening or scarring of lung
tissue because of excess collagen. Pulmonary fibrosis occurs in a small
percentage of people with systemic sclerosis.
Pulmonary hypertension--Abnormally high blood
pressure in the arteries supplying the lungs that may be caused by a
number of factors, including damage from fibrosis.
Raynaud's phenomenon--A disorder of the small blood
vessels of the extremities, causing coldness and reduced blood flow. In
response to cold or anxiety, these vessels go into spasms, causing pain,
the sensations of burning and tingling, and color changes.
Rheumatic--An adjective used to describe a group of
conditions characterized by inflammation or pain in the muscles, joints,
and fibrous tissue. Rheumatic diseases or disorders can be related to
autoimmunity or other causes.
Sclerodactyly--The hard, shiny appearance of
fingers caused by excess connective tissue buildup. This is a common
feature of scleroderma, but it may also occur in other conditions.
Systemic condition--A condition involving the body
as a whole, as opposed to limited conditions that affect particular
parts of the body.
Systemic lupus erythematosus--A systemic rheumatic
disease that occurs predominantly in women and is characterized by
autoimmune activity, a facial rash across the bridge of the nose and
cheeks, Raynaud's phenomenon, joint pain and swelling, fever, chest
pain, hair loss, and other symptoms. Many of its symptoms overlap with
those of scleroderma.
Telangiectasia--Small red dots, usually on the face
and hands, resulting from tiny blood vessels showing through the skin's
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