What is scleroderma?

1. What is Scleroderma?
2. What are the symptoms of Scleroderma, and how are they treated?
3. Managing Scleroderma
4. Progress through research

 

Understanding & Managing Scleroderma

1. What is scleroderma? 

Scleroderma, or systemic sclerosis, is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases.
The word “Scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin. Hardening of the skin is one of the most visible manifestations of the disease. The disease has been called “progressive systemic sclerosis,” but the use of that term has been discouraged since it has been found that Scleroderma is not necessarily progressive. The disease may take several forms which will be explained later. There is also much variability among patients.
Scleroderma is a disease whose symptoms may be visible, as is the case when the skin is affected, or the symptoms may be invisible, as when internal organs are affected.
What Scleroderma is not
Scleroderma is not contagious, it is not infectious, it is not cancerous or malignant, and it is usually not hereditary.
How serious is Scleroderma?
Any chronic disease can be serious. The symptoms of Scleroderma vary greatly from individual to individual, and the effects of Scleroderma can range from very mild to life-threatening. The seriousness will depend on what parts of the body are affected and the extent to which they are affected. A mild case can become more serious if not properly treated. Prompt and proper diagnosis and treatment by qualified doctors may minimize the symptoms of Scleroderma and lessen the chance for irreversible damage.
Who develops Scleroderma, and when?
It is estimated that there are over 5000 persons with Systemic Scleroderma in Australia. True prevalence is believed to be much higher as the complex nature of this illness can often lead to mis-diagnosis. Statistically, approximately three to four times more women than men develop the disease. Scleroderma can develop and is found in every age group from infants to the elderly, but its onset is most frequent between the ages of 25 to 55.
Factors other than sex, such as race and ethnic background, may influence the risk of getting Scleroderma, the age of onset, and the pattern or severity of internal organ involvement. The reasons for this are not clear. Although Scleroderma is not directly inherited, some scientists feel there is a slight predisposition to it in families with a history of rheumatic diseases.
What causes Scleroderma?
The exact cause or causes of Scleroderma are still unknown, but scientists and medical investigators in a wide variety of fields are working hard to make those determinations.

What is known about the disease process in Scleroderma is that it involves three features:
1.An overproduction of collagen
2.An autoimmune process
3.Blood-vessel damage.

Collagen is the major protein portion of the connective tissue of the body, which includes the skin, joints, and tendons, and parts of internal organs. When there is an overproduction of collagen, thickening and hardening of the affected areas takes place, often interfering with the normal functioning of those parts.
One theory is that the immune system is activated inappropriately, causing abnormal levels of cytokines to be produced. These, in turn, mount an attack not against a foreign invader but against the body’s own healthy tissues, stimulating an overproduction of collagen.

Another theory, the “vascular theory,” concerns blood vessels. Damage to the blood vessels, especially the small ones, is typical in Scleroderma. Injury to blood vessels causes them to constrict and stiffen and leads them to overreact to cold or stress. These reactions can cause further damage to the vessels themselves and to the organs, which they supply. There may also be a connection between the build up of excess collagen and blood vessels, the processes which take place, and their significance for prevention and treatment.

Are there different forms of Scleroderma?
There are two major classifications of Scleroderma
Morphea or Localized Scleroderma and Systemic Scleroderma (SSc). Systemic Scleroderma is further divided into Limited and Diffuse.

Localized Scleroderma

Morphea
In this condition there are localised patches of thickened skin. The skin affected often appears waxy and may have red or brown colour. These changes are usually found in only a few places on the skin or in the muscles, and only rarely spread elsewhere. The patches may enlarge or shrink, and often disappear spontaneously. Morphea usually appears between the ages of 20 and 50, but can also be seen in young children.

Linear This is a form of localized Scleroderma which frequently starts as a streak or line of hardened, waxy skin on an arm or leg or on the forehead. Sometimes it forms a long crease on the head or neck, referred to as “en coup de sabre” because of its resemblance to a sabre or sword wound. Linear Scleroderma tends to involve deeper layers of the skin as well as the surface layers, and sometimes restricts the movement of the joints that lie underneath. Linear Scleroderma usually develops in childhood. In children the growth of the involved limb may be affected.


Systemic Scleroderma (Systemic Sclerosis)


The changes occurring in systemic Scleroderma may affect the connective tissue in many parts of the body. Systemic Scleroderma can involve the skin, oesophagus, gastrointestinal tract (stomach and bowels), lungs, kidneys, heart, and other internal organs. It can also affect blood vessels, muscles, and joints. The tissues of involved organs become hard and fibrous, causing them to function less efficiently. The term systemic sclerosis indicates that “sclerosis” (hardening) may occur in the internal systems of the body. There are two major recognized patterns that the illness can take, limited or diffuse disease. The extent of skin involvement is used to divide the patients into these two groups. In general, the skin involvement in Scleroderma begins at the fingers and spreads up the arms. Some thickening of the skin of the face is very common, and in some patients the legs are also involved. In the legs, the skin thickening tends to begin on the foot and spread up the leg. Patients are classified as limited if, in addition to the involvement of the face, there is thickening of the skin from the hands only to the elbows, and in the legs, if the thickening extends from the foot only as far as the knee. Patients are classified as diffuse if there is more extensive spread of the skin thickening, that is, the skin of the upper arms, thighs or trunk is involved.

Limited Scleroderma
Limited Scleroderma usually causes Raynaud's phenomena and hardening of the skin in the hands. There may be some changes in the facial skin and as indicated above, occasionally there is thickening of the skin on the forearm and lower leg. Oesophageal problems are common. Although, as indicated in Table 1, occasionally other internal organ involvement does occur, it is important to realise that this involvement is often very mild and may occur only after many years of the disease. The onset of limited Scleroderma is often very slow, and any progression of skin involvement is also very slow occurring only after many years. The outlook for limited Scleroderma is generally very good. About 70% of people with Systemic Scleroderma have Limited Scleroderma with the other 30% having the more severe Diffuse form."

Diffuse Scleroderma
Diffuse Scleroderma affects the skin not only on the hands and forearms but it can also affect the skin on the trunk, upper arms and thighs. Patients with this condition often have a more systemic illness with the Scleroderma process potentially affecting many other organs and tissues. This type of Scleroderma often requires more intensive treatment, and some patients with this type have a serious disorder. Diffuse Scleroderma generally has a fairly rapid onset of disease with the skin thickening spreading rapidly over a few months. However skin thickening can remit after several years with little long-term damage, assuming the patient did not have any significant problems in the first few years of the disorder when it was active.

CRESTIs another name sometimes used to describe a subgroup of patients with Scleroderma. This term was more commonly used in the past but nowadays it is used less commonly as classification into limited and diffuse has been found more useful in predicting long term outlook. CREST is the acronym for the clinical combination of Calcinosis, Raynaud's phenomena, oEsophageal problems, Sclerodactyly (stiff fingers) and Telangectasia (small dilated red vessels in the skin of the hands or face). Most patients with CREST have limited Scleroderma.
Although most patients can be classified as having either diffuse or limited disease, different people may have different symptoms and different combination of symptoms of the illness.

How is Scleroderma diagnosed?
Diagnosis of Scleroderma may be very difficult, particularly in its early stages. Many of its symptoms are common to, or may overlap with, those of other diseases, especially other autoimmune connective-tissue diseases such as rheumatoid arthritis and lupus. Different symptoms may develop in stages over a very long period of time, and few people with Scleroderma experience exactly the same set of symptoms and effects.
While Scleroderma can often be suspected from its more visible symptoms, no single test can prove its presence. The diagnosis is usually made by your doctor through a combination of the following: the medical history, including past and present symptoms; a thorough physical examination; and findings from a variety of laboratory tests and other studies. In making the diagnosis, it is important not only to confirm the presence of Scleroderma, but also to determine its extent and severity, particularly with regard to the involvement of internal organs.
Diffuse and limited Scleroderma can sometimes be differentiated by the presence of different antibodies, called anti-nuclear antibodies (ANA) in the blood. For example, anti-Scl-70 is frequently associated with diffuse Scleroderma whereas anti-centromere is usually indicative of limited Scleroderma.

Back to top

2. The symptoms of scleroderma, and how they are treated This section describes the most common symptoms of Scleroderma and some of the treatments being used to control them.

Scleroderma is a complex disease with many possible symptoms that can affect many parts of the body. Most people only develop a few of the symptoms mentioned. Each patient is different in terms of symptoms and severity. Typically, the symptoms may also vary over time with periods of improvement and worsening. It is not possible here to describe all of the symptoms or all of the methods being used in the management of Scleroderma. A great variety of treatments and medications have been tried over the years and new ones are constantly being tested. Doctors experienced in Scleroderma should be consulted regarding any symptoms or treatments mentioned here, as well as for any other symptoms that may be experienced.
Even though Scleroderma is not curable, many of the symptoms can be improved with medication or lifestyle changes.
Raynaud’s phenomenon
Raynaud’s phenomenon is the most common early symptom of systemic Scleroderma. It is present at one time or another in about 90% of patients. It is most obvious in the fingers and toes but can also involve the ears, nose, and tip of the tongue. In Raynaud’s phenomenon, the blood vessels constrict or narrow in response to cold or to emotional upset and stress. The resulting disturbance in circulation of the blood causes a series of colour changes in the skin: white, blanched, or pale, when circulation is reduced; blue as the affected part loses oxygen from decreased blood flow; and then red or flushed as blood flow returns and the affected skin re-warms. Finally, as the attack subsides and the circulation returns to normal, usual skin colour is restored. In the “white” or “blue” stages, sensations such as tingling, numbness and coldness may be felt. In the “red” stage, a feeling of warmth, burning, or throbbing may be noted. In some people the Raynaud’s attacks are painful.
Many common-sense preventive measures can be taken by those susceptible to Raynaud’s phenomenon. The most obvious is minimizing exposure to cold, such as outdoor weather, air conditioning, or reaching into a refrigerator or freezer. Keeping warm is very important. It is important to not only protect your hands and feet from cold but to also keep the whole body warm.
A warm bath or shower, or a heating pad or hot water bottle on the back, may relieve an attack better than just warming the hands. Avoidance of emotional upset and stress is helpful but not always possible. Relaxation techniques of various kinds, whether self-taught or learned through training courses, have proven effective for some people in managing stress.
Smoking definitely worsens Raynaud’s phenomenon. For this and other reasons, people with Scleroderma should not smoke.
Your doctor may suggest a number of different medications to prevent, reduce the frequency, or minimize the effects of Raynaud’s phenomenon. However, these medications can cause side effects such as palpitations, facial flushing, headaches, light-headedness, swelling of ankles and constipation, which might not allow the patient to take a large enough dose to control their Raynaud's. There are now over a dozen medications to improve circulation, and it is not possible to list them all here.
Raynaud’s phenomenon is not confined to people with Scleroderma. It is also seen in lupus, rheumatoid arthritis, and other connective tissue diseases. In addition, many healthy people have Raynaud’s phenomenon without any other illness. In this situation, it is called “primary Raynaud’s disease.”

Swelling or puffiness of the hands
Swelling is another typical early symptom of Scleroderma, and this may be especially noticeable upon awakening because of muscle inactivity overnight. The skin of the fingers may look full and sausage-like, making it difficult to close the hand into a fist. Exercising the fingers and toes is helpful. Your doctor may recommend medications to reduce inflammation.

Pain and stiffness of the joints
Symptoms of pain, stiffness, swelling, warmth or tenderness may accompany the arthritis-like joint inflammation that frequently occurs in Scleroderma. Muscle pain and weakness are other important symptoms. Anti-inflammatory prescription drugs can be helpful in reducing pain in this situation. However because of their side effects they are not suitable for all patients
A physiotherapist can develop an exercise plan after consultation with your doctor.Skin disorders Skin thickening.


Hardening and thickening of the skin give Scleroderma its name (“hard skin”). There are no proven treatments as yet to universally prevent or alter the course of the skin changes in Scleroderma. It is however important to note that some patients with diffuse disease often experience a dramatic softening of their skin after several years.


Skin ulcerations.
Sores, especially on the fingertips, are a common symptom of systemic Scleroderma. They may be very slow or difficult to heal because of poor circulation. These sores or ulcerations may also occur on the knuckles, elbows, toes, or other sites of the body where the skin is especially tight or stretched. The affected area should be kept warm to increase blood flow, and scrupulously clean to avoid infection. It is very important to consult your doctor early if you suspect an ulcer is infected as early treatment can prevent further damage. Appropriate dressings can aid healing and relieve pain.


Calcinosis.
This condition is characterized by deposits of calcium in the skin, which may be painful. The calcium deposits may occur just below the skin surface in the form of hard lumps or nodules. They may break through the skin, becoming visible as chalky white material, and may become infected. Care should be taken not to bump or injure affected areas. Warm-water soaks may be helpful. Antibiotics may be prescribed to prevent or control infection. In severe cases, surgery to remove calcium deposits may be required.


Telangiectasia.
This abnormality consists of the dilation of small blood vessels near the surface of the skin, which become visible as small red spots, usually on the fingers, palms, face, and lips. The spots usually fade with pressure, but turn red again when the pressure is released. These spots are generally not harmful. Special cosmetics may be used to mask the spots or to reduce their visibility.


Dry skin.
Excessive dryness of the skin may lead to skin breakdown and ulcerations. Excessive bathing and hand washing should be avoided, and rubber gloves worn to avoid direct contact with household detergents. Keeping the skin moist and well-lubricated is important to avoid complications from dry skin. Bath oils and moisturizing soaps such as Neutrogena® and Dove™ are preferable to harsh soaps which dry out the skin. Frequent use of moisturizing skin creams containing lanolin or sorbelene is advisable. A room humidifier may be helpful during the winter months.


Itchy skin.
If moisturizing creams do not work, your doctor may prescribe a topical cortisone cream to be rubbed on the skin to relieve itching. Antihistamines tablets have been effective for some people.


Other skin symptoms.
There may be a decrease in hair over affected areas of the skin, as well as a decrease in the ability to perspire. In addition, there may be an increase in pigment (which looks like a skin tan) or a spotty loss of pigment.


Sclerodactyly and joint contractures
Sclerodactyly means simply “hard skin of the digits”; that is, of the fingers and toes. It generally occurs after initial swelling has subsided. It is characterized by shiny, tight skin of the fingers. Affected digits may be difficult to move, and they may become fixed in a bent or flexed position called a “contracture” or a “flexion contracture.” Tightening and hardening of the skin and of the tissues surrounding the joints can cause decreased movement of the wrists, elbows, and other joints.
An occupational therapist can develop an exercise plan after consulting with your doctor. He/she may also provide devices to help perform common personal care and household tasks more easily.


Digestive system and gastrointestinal tract problems
People with systemic Scleroderma may develop abnormalities of the digestive system and gastrointestinal tract from the mouth to the anal canal. The overproduction of collagen typical of Scleroderma can cause thickening and fibrosis (or scarring) of the tissues. This can result in weakened muscles, and lead to the abnormally slow movement of food (“dysmotility”) in the digestive process.


Oesophageal dysfunction.
Food travels from the mouth and throat into the stomach through a tube called the oesophagus. Normally, the lower oesophageal sphincter or valve acts as a gate which opens to permit food to enter the stomach and then closes promptly to prevent food from coming back up. In Systemic Scleroderma, the gate does not close properly and the result is a backwash of acid and a burning sensation (“heartburn”) as food and acid return into the oesophagus. The acid may also injure the lining of the lower portion of the oesophagus, causing scarring and a narrowing (“stricture”) of the tube.
Acid production can be reduced, and the problems of acid reflux and heartburn lessened, by avoiding (to the extent possible) alcohol, greasy or fatty foods, spicy foods, chocolate, tobacco, and caffeine. However many people, perhaps the majority, will require a medication to decrease the acidity of the stomach so that any fluid washing back into the oesophagus will not be harmful to it.
The force of gravity helps to keep food and acid in the stomach; therefore, an upright position after meals is helpful. Other common-sense measures to prevent acid from coming up into the oesophagus include eating smaller and more frequent meals, not eating for several hours before bedtime, and elevating the head of the bed six to eight inches with wooden blocks. Being overweight can make reflux worse, and wearing girdles or other tight-fitting garments should be avoided.

Swallowing difficulties.
Abnormally slow movement of food and narrowing of the oesophagus may cause swallowing difficulties. Eating slowly and chewing thoroughly are important. Swallowing and digesting are made easier by eating softer foods (many foods can be prepared in a blender) and avoiding foods which tend to stick in the throat. If the oesophagus has narrowed significantly, you may need to have the oesophagus dilated periodically to permit easier swallowing.


Diarrhea.
In systemic Scleroderma, there can be damage to the muscles of the small bowel (small intestine). The weakened muscles do not work effectively to push food through the bowel. Simply put, things “sit” rather than move well. One consequence can be an overgrowth of bacteria, leading to diarrhea. There may also be a bloated, distended feeling and some pain if the bowel is stretched. Another effect is that the nutrients of food remain in the bowel instead of being absorbed into the body. This condition is called “malabsorption,” and it may lead to weight loss and stool abnormalities.


Constipation.
Weak or scarred muscles in the colon wall make it difficult for the bowel to work well, resulting in constipation or other abnormalities of the colon. Maintaining a diet high in fibre, and drinking at least six to eight glasses of fluids daily, especially water, will help reduce constipation. Fresh fruits and vegetables are natural laxatives. Exercise also helps to keep bowel movements regular. Your doctor may also recommend stool softeners and bulking agents like Metamucil or Fybogel.

Sjogrens Syndrome 

Sjögren’s Syndrome (dry eyes, dry mouth) is characterized by a decrease in secretions of the tear glands and the salivary glands, which provide lubrication for the eyes and mouth. The unusual dryness of the eyes resulting from this condition can lead to serious irritation and inflammation. Excessive dryness of the mouth may lead to difficulties in swallowing and in speaking, a pronounced increase in tooth decay and cavities, and a reduced sense of taste. The lack of secretions in Sjögren’s Syndrome may also involve the vagina and other areas of the body.


Dry eyes may be lubricated by the frequent use of artificial tears and ophthalmic ointments. Regular visits to the ophthalmologist are important. The mouth should be kept as well-lubricated as possible by sipping fluids throughout the day (a plastic squirt bottle filled with water may be useful), and by chewing sugar-free gum or sucking sugar-free sour candy to stimulate salivary activity. Artificial saliva is also available. (See next section for preventive dental care.)
Lubricants such as KY gel can help to moisten the vagina and facilitate sexual relations. Avoiding pantyhose and other tight-fitting clothing may help to reduce irritation and prevent infection. Choose cotton rather than nylon underwear.


Oral, facial, and dental problems
People with Scleroderma may experience a general tightening of skin over the face. The opening of the mouth may be decreased in size (“microstomia” or “small mouth”), making lip and mouth movements as well as oral hygiene difficult.
The best approach to treatment is by means of facial grimacing and mouth stretching exercises daily.
Preventive dental care through regular flossing and brushing of the teeth and gums after each meal is very important. Regular dental visits are also important to help prevent dental caries. Your dentist can also recommend a program of good oral hygiene. Floss holders, pump toothpaste tubes, and built-up handles on toothbrushes can help people with hand impairment. (These measures are equally important for those with Sjögren’s Syndrome.)


Kidney involvement
Kidney or renal involvement in Systemic Scleroderma may be mild or very serious in nature. Early signs of kidney involvement may include mild hypertension (high blood pressure), protein in the urine, and blood test abnormalities. “Renal crisis,” a highly dangerous complication of Systemic Scleroderma, may occur quite suddenly. Its most important warning signal is an abrupt rise in blood pressure. Renal crisis occurs in about 10% of people with diffuse scleroderma and is most common in the first 4 years of their disease. It is very uncommon to see renal crisis in people with Limited Scleroderma.
Symptoms include, new severe headache, visual disturbances, shortness of breath, chest pain or discomfort, or mental confusion. Unless treated promptly, renal crisis leads to kidney failure, a condition in which the kidneys lose their ability to eliminate waste products from the body. People with Scleroderma are advised to have their blood pressure and kidney function monitored at regular intervals.
People may recover successfully from renal crisis, but only if the problem is recognized and treated quickly.


Lung involvement
The lung can be affected in Scleroderma by 3 different processes:
1.Build-up of collagen thickens lung tissue and causes fibrosis or scarring, making the transport of oxygen into the bloodstream more difficult. This is called pulmonary fibrosis or Interstitial Lung Disease (ILD)
2. Pulmonary Arterial Hypertension (PAH), a state of increased resistance to blood flow through the lungs, can result from damage to blood vessels, and may lead to increased strain on the heart resulting in heart failure.
3. Respiratory muscle weakness may decrease lung function.
Symptoms of lung involvement include shortness of breath, a decreased tolerance for exercise, and a persistent cough. The physician may order a chest x-ray and/or CT scan of the chest, an echocardiogram (ultrasound of the heart), or special breathing tests (pulmonary function tests) to detect or confirm lung involvement and to determine which of these 3 processes is responsible for the symptoms
In the very early stages of lung fibrosis, medications may be given to decrease the inflammation which is thought to lead to lung scarring. Recent trials have shown that patients with severe and progressive lung fibrosis may gain some benefit from immunosuppressive drugs such as Cyclophosphamide.
It is important for people with Scleroderma to take whatever measures are within his or her control to avoid further damage to the lungs. It is essential to avoid smoking, a major cause of lung disease.
Pulmonary Arterial Hypertension (PAH) can now be treated with special medications targeted at dilating or opening up the blood vessels of the lungs, and possibly changing the underlying nature of the disease. This is one complication of Scleroderma for which new medications have proven successful. In Australia there are currently two medications are approved for the treatment of PAH. New treatments are under development and may be come available in the next few years.


Heart involvement
If the heart muscle becomes thickened and fibrous scar tissue accumulates, the force of heart contractions may be decreased, which may ultimately result in heart failure. Spasm of the coronary arteries (the main blood vessels to the heart), may cause chest pain and, rarely, lead to a heart attack. The spasm appears similar to that involving the fingers in Raynaud’s phenomenon. Inflammation of the outer heart lining (pericarditis) may cause pain and accumulation of fluid around the heart. An irregular heartbeat may also be experienced. These conditions require careful evaluation and treatment by your specialist.

Non-specific symptoms
The person with systemic Scleroderma may experience a variety of non-specific symptoms, including fatigue (ranging from mild to severe), lack of energy, generalized weakness, weight loss, and vague aching of muscles, joints, or bones. Treatments or medications recommended by your doctor will depend on his/her evaluation of the causes of these symptoms.

Back to top

3. Managing Scleroderma
The reader may be aware of, or learn about, other forms of treatment that have been used or are proposed for use in managing Scleroderma in addition to those discussed in this booklet. Scleroderma is a difficult disease to study because of its variable nature, its prolonged course, and the relatively small number of persons affected by it. Under these circumstances, it is difficult to conduct scientifically sound studies proving the value of a particular drug or treatment. Therefore, your doctor must often make decisions about treatment based on incomplete information. He/she must weigh the possible benefits against the potential risks or side effects. Further investigation will ultimately determine which treatments are beneficial and which treatments are not.


The course of Scleroderma
Scleroderma has many forms and a number of different symptoms that may present themselves singly or in combinations at various times throughout the course of the disease. Some symptoms develop with relative suddenness; others take years to develop. The exact course the disease may take is unpredictable, and the prognosis will vary from individual to individual. Systemic Scleroderma is a chronic, life-long disease. At present there is no known cure, but as with other chronic diseases there are many means available to control or manage its symptoms. It is helpful to keep Scleroderma in perspective. Many persons with the disease have few or minimal symptoms and are able to lead a normal or nearly normal life.
There may be periods of time when the person with Scleroderma will be free of troubling symptoms and feel well. At other times, he or she may feel quite ill. Spontaneous improvements may occur. The skin, in particular, sometimes softens and becomes more pliable after a number of years. Spontaneous remissions, times when symptoms may actually disappear, may also occur and may continue for long periods. The person with Scleroderma should be cautious about attributing such improvements or remissions to a particular treatment, diet, or so-called “cure.”

Being alert to symptoms
Information presented here describes many symptoms although each person with Scleroderma usually develops only a few of them. Its purpose is not to overwhelm people with Scleroderma or those who suspect they may have the disease but to provide them with useful information on what to look for, what may occur during the course of the disease, and some of the things that can be done if symptoms do develop.

Learning to recognize early symptoms of disease activity can lead to earlier detection and diagnosis of Scleroderma and to prompt initiation of treatment. Some of the more promising medications in current use are slow-acting and the sooner treatment is begun, the better the results may be. If one has already been diagnosed as having Scleroderma, it is especially important to watch for and report to the physician new or changed symptoms. Early treatment may prevent symptoms from worsening and may decrease the chance of permanent tissue or organ damage.

In being alert to symptoms, it would be a mistake to assume that every symptom or condition that develops is necessarily related to, or the result of, Scleroderma. People with or without Scleroderma do suffer accidents, contract infectious diseases, and develop other illnesses. Your doctor can help to distinguish what is related to Scleroderma and what is not, and recommend appropriate treatment.

Common-sense measures to help manage Scleroderma
• Avoiding over-fatigue by “taking it easy” and getting sufficient rest; knowing your own limits does not indicate you are “lazy.”
• Learning to control and minimize stress.
• Eating well-balanced meals and maintaining a sensible weight.
• Practicing habits of good hygiene, especially of the skin, teeth, gums, and feet (including the wearing of cushioned and well-fitted shoes).
One key measure, mentioned previously, is avoiding smoking. The health risks of smoking are well known but frequently ignored. It is particularly dangerous to persons with Scleroderma because it can have effects on blood circulation and lung function.

The emotional aspects of Scleroderma
A common reaction to being told that one has a disease such as Scleroderma is “Why me?” It is not known why some people develop the disease and others do not. One does not bring Scleroderma upon himself or herself; therefore, one need not feel guilty or responsible for the illness.
A person newly diagnosed with Scleroderma may feel alone and uncertain about where to turn for help. He or she may experience a number of other feelings and emotional reactions from time to time, including initial shock or disbelief, fear, anger, denial, self-blame, or guilt, grief, sadness, or depression. Family members may have similar feelings.
Feelings in themselves are neither good nor bad.... one simply “has” them. Sharing them with family and friends or with others who have had similar experiences can be helpful


Building a health and support network
Participating actively in one’s own healthcare is of prime importance to the person with Scleroderma. It is equally important to cooperate and communicate effectively with the doctor who is managing the disease. While these two—the person with Scleroderma and the doctor—are the focal point of the management “team,” many other people and resources can be enlisted to form a health and support network.
Family and friends can provide emotional support for the person with Scleroderma, encourage him/her to follow the recommended treatment program, and assist in carrying out activities that he/she finds difficult.
The health team begins with your doctor, but can include many other health professionals such as other medical specialists, nurses, physiotherapists and occupational therapists, and psychologists or others trained in counselling.
Joining a Scleroderma support group, such as one affiliated with Scleroderma Australia, enables the person with Scleroderma to meet and to exchange information with others who have similar problems, as well as to learn more about Scleroderma.
The extent of the health and support network is limited only by the imagination and resourcefulness of those helping to create it.

Back to top

4. Progress through research
Is there hope and help, for the person with Scleroderma?
Emphatically, yes!
There are many treatments and medications available now to help the person with Scleroderma, and more and more doctors are becoming interested in the disease.
Researchers throughout the world are intensifying their efforts to understand the nature and discover the cause of Scleroderma, to find better means of prevention and treatment, and to find a cure. These efforts reflect the increased interest in all of the connective-tissue and rheumatic diseases.
Research has already resulted in better laboratory tools to detect the early stages of Scleroderma and in improved methods of measurement to evaluate disease progression and the results of treatment. Various animal models of Scleroderma have been developed.
Investigators are currently studying the role of the immune system in Scleroderma, exploring the relationship between blood vessel changes and fibrosis, and seeking “markers” to identify the various forms and subsets of Scleroderma. These are just a few of the many studies in progress.
Scleroderma poses many questions. Answers may come from a variety of medical and scientific fields, or from totally unexpected sources… but they will come!

Back to top 

 

Download the reg form hereBecome a Member

1. Download our registration form (left)

2. Be included in the newsletter

Link to alternative payment options

Popular

Please note: The new phone number for the Scleroderma Association of Queensland is 07 32773460. Our postal has changed too; it is PO Box 316, SALISBURY Qld. 4107. The new physical address of our office is 124A Evans Road Salisbury. If you would like to come and browse through some books in our library or have a chat or help, please visit on Wednesday between 9:30 and 12 midday.