Scleroderma is a rare, chronic autoimmune disease that causes skin to thicken and tighten. For some patients, it also causes life-threatening damage to internal organs. Although there's no cure for the disease, its impact on people's lives can be reduced.
The systemic forms of scleroderma
cause fibrosis (scar tissue
) to be formed in the skin and/or internal organs. The fibrosis eventually causes the involved skin or organs to harden, which is why scleroderma is popularly known as the "disease that turns people into stone."
There are two main types of scleroderma, localized
. The systemic forms can affect any part of the body (skin, blood vessels, and internal organs.) They are also referred to as "systemic sclerosis
" plus other terms such as diffuse, limited, CREST
, and overlap. When systemic scleroderma affects the skin, it can cause the skin - most commonly on the hands and/or face - to harden. With affecting the blood vessels, it can cause Raynaud's phenomenon
. When it affects the internal organs, it may cause disability or even death.
The localized forms are morphea and linear. They affect only the skin (and sometimes the underlying tissues) but do not affect the internal organs, or reduce one's life expectancy in any way.Diffuse
scleroderma is when skin tightening also occurs above the wrists or elbows. Limited
scleroderma is when skin involvement is limited to the hands (although the face and neck may also be involved.) CREST
stands for Calcinosis, Raynaud's
, Sclerodactyly, and Telangiectasia
. CREST may occur alone, or in combination with any other form of scleroderma (or even other autoimmune
diseases) such as CREST with limited scleroderma, or CREST with lupus. If a scleroderma patient also has any other autoimmune disease
(such as lupus, rheumatoid arthritis
, etc.) it is referred to as overlap
; as in "Diffuse scleroderma in overlap with lupus." Scleroderma symptoms can also be a part of Mixed Connective Tissue Disease (MCTD), or Undifferentiated Connective Tissue Disease (UCTD).
When any form of scleroderma (either localized or systemic) occurs in children, it is also called Childhood Scleroderma or Juvenile Scleroderma.
Incidence; Causes and DevelopmentScleroderma
afflicts an estimated 150,000 to 500,000 Americans, primarily females who are 30 to 50 years old at onset. The overall occurrence is 30 people per 100,000 and the ratio of women to men is about four to one. Average age at diagnosis is significantly younger for blacks than for whites, and black patients are almost twice as likely to have diffuse disease than whites. Median survival is 11 years. [Wiley InterScience. Arthritis Rheum. 2003 Aug;48(8): pp.2246-55
The cause of scleroderma
is unknown. Some cases of scleroderma have been linked to chemical exposures.
Diagnosis and Tests
Proper diagnosis of scleroderma
is often long and difficult, since it is a rare disease which few doctors are well-versed in, and in the early stages it may resemble many other connective tissue diseases, such as systemic lupus erythematosus, polymyositis, and rheumatoid arthritis
, just to name a few.
Often the diagnostic uncertainty and frustration takes a huge toll on the psychological well-being of the patient, who sometimes describe their journey to diagnosis as being by far the most difficult part of their illness. Many other diseases may have a scleroderma-like appearance. In these diseases, abnormalities of nail folds, visceral involvement and immunological changes are usually different from those observed in systemic scleroderma
. [PubMed. Rev Prat. 2002 Nov 1;52(17): pp.1884-90
Treatment and Prevention
At present, there are no proven treatments or cure for scleroderma
of any form. However, there are effective therapies for many of the symptoms.
There are several "natural" things you can do for systemic scleroderma
- Avoid attacks of Raynaud's as much as possible. Try to learn various ways to prevent Raynaud's and to restore circulation. Then respond to every attack of Raynaud's as if it were a medical emergency, because even one prolonged attack can lead to gangrene, and possibly even amputation.
- One thing which will give you the greatest odds of living longer with systemic scleroderma, is to monitor your blood pressure at home every day or two. One of the most life-threatening, yet treatable, complications of systemic scleroderma (including CREST) is sudden kidney failure. If your blood pressure is elevated over a certain point (to be determined by your doctor) you should call your doctor immediately. You should also ask your doctor to take your blood pressure on every office visit.
- Stress can play havoc with your immune system, as well as trigger and worsen attacks of Raynaud's. Alternative therapies that help you deal more effectively with stress, such as biofeedback and yoga, may improve your symptoms.
- Strive for moderation in everything - just enough but not too much exercise, good sleep every night with sufficient rest breaks during the day, and well-balanced, regular meals.
- In general, most doctors recommend that scleroderma patients follow a normal, well-balanced diet, without the addition of any herbs, vitamins, or minerals. Do not cut out any food groups from your diet, nor go on any "cleansing" diets, since this may accelerate the disease process.
- Don't go on any diets that eliminate certain food groups.
- Avoid high-protein diets because they are very hard on the kidneys.
- For relief of symptoms such as Raynaud's and heartburn, you may want to particularly avoid:
- caffeine (can trigger Raynaud's and heartburn)
- chocolate (can trigger Raynaud's and heartburn)
- peppermint (can trigger heartburn)
- ginger (can trigger heartburn)
Many herbs and over-the-counter medications can interact with prescription medications, and many of them make scleroderma symptoms worse. Even simple over-the-counter cold remedies often have ingredients that can trigger Raynaud's or heartburn in systemic scleroderma patients.
The immune system does not need to be improved in scleroderma, because it is already overactive (and not underactive, as in AIDS
.) Doing things that enhance the immune system can actually make scleroderma worse in some cases, as it gives the body more power to attack itself.
Research shows that Raynaud's can cause an abnormal processing of certain vitamins and minerals, leading to worsening scleroderma. So excessive intake of any vitamin is to be avoided.
In the past, only the worst cases of scleroderma
were diagnosed, thus leading to the widespread belief that systemic scleroderma was always progressive and fatal. Now that the full range of types and severity of scleroderma are being recognized and diagnosed, there is increasing awareness that many people have a milder type of illness with a much better long term outlook.
References and Further InformationContent on this page reprinted with permission from the nonprofit International Scleroderma Network (ISN) at sclero.org, which is the stellar world resource for scleroderma and related illnesses, like Raynaud's and pulmonary hypertension. All rights reserved.