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LEARNING ABOUT SCLERODERMA
Scleroderma, also known as systematic sclerosis, is an uncommon but not rare disorder. Many patients, when told they have this problem, find it difficult to find further information on this condition. In particular, it is common for scleroderma to be portrayed "in the wrong light" without an understanding of the type of scleroderma that they have, and also the extent of the condition in their particular case. This information is extremely important as it provides the patient with an understanding of their outlook - what the disease on average might do to the patient and how the disease will affect their future.
An appropriate understanding of scleroderma, especially the way it affects the individual with the problem, is the first pre-requisite for gaining self management skills. The management of any chronic illness requires an interaction between the patient, their doctor, other health professionals and their family and friends.
Even in the recent past, scleroderma was regarded as a disease with the potential to cause considerable disability, and one that might even cause death. However, the perspective on scleroderma has changed considerably. It is now recognised that it is far more common than it was believed to be, and milder form manifest in many people who never develop more serious disease. Furthermore, whilst a cure is not yet available, a large number of treatment options are now available for the various manifestations of the condition. There is no doubt that the outlook for someone with scleroderma diagnosed nowadays is considerably better than ever before.
Nevertheless, scleroderma needs to be taken seriously and
patients are advised to have reviews and follow appropriate medical advice carefully.
What is scleroderma?
Who suffers from scleroderma?
It is known that the immune system in patients with scleroderma is activated. One example of this is the presence of autoantibodies in most patients with scleroderma.
Antibodies are proteins which are normally directed against foreign substances such as bacteria. They are a normal part of the body's immune system and are an essential defence against infection. However, if these antibodies develop against parts of your own body (autoantibodies), they can cause disease.
In addition other parts of the immune system are activated in this disease. Lymphocytes, which are the cells of the immune system, can be found in increased numbers in tissues affected by scleroderma. It is likely that they release chemicals which initiate the damaging process.
In many small blood vessels in patients with scleroderma, the lining cells and the wall of the blood vessels thicken causing narrowing of the lumen (diameter) of the blood vessel. In addition, the ling cells become more activated making them more sticky. This results in a tendency to increased clotting of the blood within the blood cells.
The combination of these two processes leads to a reduced blood flow through the small blood vessels, thus reducing the blood supply to the affected tissues.
These processes are partly responsible for the Raynaud's phenomena which is very common in patients with scleroderma. In addition to this, more fixed narrowing of the vessels, contraction of the muscle in the walls of small arteries in response to cold or emotion, causes further reversible narrowing of the blood vessels.
3. CONNECTIVE TISSUES
Substances released by the immune system can also activate
cells called fibroblasts. These cells produce collagen which is normal structural protein
or building block of the connective tissue throughout the body. In people with
scleroderma, some of the fibroblasts become overactive and produce excessive amounts of
collagen. The body is unable to clear this excessive collagen and it therefore
accumulates, causing excessive thickening and hardening of the tissues affected. This is
most easily demonstrated in the skin of patients with this condition and is the cause of
the characteristic swelling and tightness that develops. The process can occur in other
tissues in the body.
Is scleroderma becoming more common?
What are the different types of 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 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 very good.
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.
Occasionally scleroderma may occur in conjunction with another connective tissue disease. For instance, some patients have features of systemic lupus erythematous or polymyositis and , yet, also have dominant features of scleroderma. Such patients are said to have an "overlap syndrome". The scleroderma part of this problem is treated the same way as if scleroderma was occurring by itself.
CREST is 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 Telangiectasia (small dilated red vessels in the skin of the hands or face). Most patients with CREST have limited scleroderma.
Morphea is a condition related
to scleroderma which is sometimes called localised scleroderma. In this condition there
are localised patches of thickened skin. Patients with this condition do not have
Raynaud's and do not have any internal organ involvement. The long term outlook is
Manifestations of scleroderma
Table 1: Manifestations of Scleroderma
However, many patients who present with Raynaud's phenomena do not have scleroderma as a cause for that problem. There are numerous causes for Raynaud's phenomena. Indeed many normal people have mild degrees of this problem. Raynaud's phenomena is a reversible skin colour change usually affecting the fingers. There are usually three phases - white to blue to red - due to cold induced spasm or narrowing of the small blood vessels of the fingers. Emotion can also cause this response. Some patients with scleroderma have cold induced blanching of the fingers or finger tips without three phases in the colours, and others have cold aggravated and persistent severe bluish discolouration of the finger tips known as acrocyanosis (blueness of the peripheries). Raynaud's phenomenon or similar problems should alert one to the possibility of scleroderma.
The next clue to diagnosis is involvement of the skin. Thickening or hardening of the skin in the fingers is characteristic of scleroderma. This may occur very slowly, and it may take some time to appreciate the changes. Subtle changes may not be initially appreciated. The Raynaud's and skin changes usually, but not always, precede other problems. Therefore occasionally other problems in other parts of the body may alert one to diagnosis. Antibody tests can be supportive of the diagnosis if skin changes are not typical. A number of autoantibodies are seen in scleroderma (see Table 2).
Guidelines to interpretation of the antinuclear antibody test (ANA) in scleroderma are as follows:
Table 2: Autoantibodies in scleroderma
Early prognostic indicators
The extent of skin involvement within one year of presentation can be a guide to predict outcome. Limited scleroderma (skin involvement of only hands, forearm, face and lower legs) has a very good survival rate compared to diffuse scleroderma (skin involvement of upper arms, thighs and /or trunk), where some patients have severe medical problems due to scleroderma.
It is extremely rare for a patient who, after one year of
disease onset has limited disease, to progress to diffuse disease.
With acceptance, there may be a need to adjust the life-style and this can involve the entire family. All are affected by the emotional impact of the disease, and keeping communication open is essential. Helping the patient adjust may require some role changing by the family. Certain tasks may be allotted differently. If problems are too difficult to resolve family counselling may be of benefit.
Patients with this condition should have regular medical supervision and should always seek medical advice about new symptoms. It is generally recommended that patients with limited disease be reviewed every 6-12months while those with diffuse disease should be reviewed at least every 3 months for the first five years of the disease then 6 monthly reviews may be adequate if there have been no complications.
Remember, attack the problems you have and don't look for new ones. Enjoy the life you have but don't expect perfect health. Many of the most important things in life were done by people who were not feeling very well.
The Scleroderma Foundation of Victoria Inc. was established
in 1979. It grew out of the efforts of a small group of people who recognised that
sufferers often felt alone and isolated.
The aims of the foundation are:
Membership is open to people with scleroderma, their family
and friends. Through meetings, newsletters and literature, members are kept informed on
the latest medical research, medical treatments and hints for daily living. The foundation
maintains contact with similar organisations throughout Australia and overseas.
For more information please contact:
Scleroderma Victoria Inc. is located at: